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A 29-year-old female presented to our orthopaedic service following a twisting injury during martial arts, where she heard her right knee pop and experienced immediate pain and swelling in her knee. Physical examination demonstrated +2 Lachman and +1 pivot in her right knee. MRI confirmed full thickness ACL tear, as well as medial and lateral meniscal pathology with a lateral meniscal tear. Arthroscopic ACL reconstruction of the patient's right knee was performed using allograft and the Bio-TransFix system (Arthrex; Naples, FL). Following drilling of the tibial tunnel and guide placements, the Bio-TransFix guide and pin were inserted and secured in the graft. An 8–10 mm IntraFix screw and sleeve construct were then inserted at this time. The graft was noted to be taut, and full extension showed no impingement both on the PCL and on the notch. There were no intraoperative complications. At six weeks after the ACL reconstruction procedure, clinical examination revealed some mild wound breakdown at the tibia and a prominence over the lateral incision, through which the cross-pin was inserted. The patient's range of motion was from 0 to 90 at this time. Lachman test was negative. The patient had no symptoms of instability. At four months after ACL reconstruction, the patient was doing well, but was still having pain over the lateral incision. An MRI of the right knee demonstrated a broken, displaced cross-pin. The proximal portion of the pin was displaced into the adjacent soft tissues and surrounded by extensive edema and a thickened iliotibial band (Figures and ). The ACL graft was intact.\nThe patient continued to have pain and elected to undergo surgery. Under general anesthesia, an incision over the previous one was performed. The IT band was split and the underlying soft tissue palpated. The Bio-TransFix pin was found to be broken and was then removed with a clamp (). Six months after the surgery, patient is doing well and has gone back to martial arts with no restrictions. Patient clinically has full range of motion in her right knee and a negative Lachman test. Patient reported complete resolution of symptoms following pin removal. |
A 24-year-old HIV positive homosexual Danish male with no other chronic diseases was admitted to the hospital with four days of febrile episodes and thoracic pain with intermittent stabbing pain in both arms. He had also had bilateral leg pain worst at night time. He denied having had any kind of rash or genital ulcers. There had been no traumas in connection to the onset of pain.\nTwo weeks before admission, the patient contacted the Department of Infectious Diseases due to a sore throat. At this point physical examination was normal including a normal examination of the oral cavity. A throat culture was negative, and the patient did not have fever. The patient had been tested positive for HIV in 2002, and antiretroviral treatment with tenofovir/emtricitabine , atazanavir and ritonavir was initiated seven months prior to admission. Prior to treatment initiation the CD4 nadir was 330 cells/μl, and the HIV-1 RNA was 44549 copies/ml. Syphilis testing was performed at treatment initiation and 7 months before admission to the hospital, both serology tests were found negative.\nOn the admission day physical examination disclosed nothing abnormal except from severe tenderness of the processus spinosus of the second thoracic vertebrae (T2). The overlying skin was without any signs of infection and no rash or genital ulcers were found. Laboratory test demonstrated a normal white blood cell count 4.8 × 109/l and C-reactive protein level of 109 mg/l. The CD4 count was 360 cells/μl, and HIV-1 RNA was 158 copies/ml. A QuantiFERON-TB gold test was negative. Eight blood cultures were done, all negative for bacterial and fungal growth. Osteitis was suspected and a bone scintigraphy was performed which revealed bilateral tibial activity as well as abnormal activity in the right side of the cranium but no spine activity was observed. Magnetic resonance imaging (MRI) of the spine revealed destructive lesions in C2 (the dens of axis) and T2 consistent with osteitis (Figure ).\nA surgical biopsy from T2 was performed, and culture from the biopsy was negative for bacterial growth. Acid-fast and Gram stain did not reveal any microorganisms. The biopsy was then analyzed for bacterial DNA. PCR for part of the 16S rRNA gene resulted in a 528 basepair fragment which by DNA sequencing and subsequent comparison to sequences deposited in the NCBI database showed sequence homology with Treponema pallidum (505 out of 507 identical bases). The bacterial identification was confirmed by PCR specific for the organism []. The pathological examination revealed an inflammatory infiltrate dominated by lymphocytes and plasmacells. Immunohistochemical analysis using polyclonal antibody against T. pallidum demonstrated several spirochetes (Figure ). Furthermore, a reactive quantitative rapid plasma reagin (RPR) syphilis serology was found to be positive with a 1:128 titer. As soon as the biopsy was obtained treatment was initiated with cefuroxime 1.5 g × 3 daily. Following the diagnosis of syphilitic osteitis, treatment was changed to Ceftriaxone 2 g × 1 daily for five weeks according to the local guidelines. The thoracic pain disappeared within the first week of treatment. A follow-up MRI of the spine performed after two weeks of treatment revealed unaltered lesions. |
A 56-year-old male with vision loss from sequential nonarteritic anterior ischemic optic neuropathy (NAION) with a complete visual field defect and a visual acuity of 1/50 Snellen in each eye () was seen acutely due to a further, painless decrease in visual acuity in both eyes. A visual acuity of light perception was noted. On examination, whitish, dense vitreal opacities partly obscuring views of deeply atrophic optic nerves and otherwise unremarkable retinas were seen. The conjunctivae were white and no anterior uveitis was seen.\nThe patient revealed that two months earlier he received intravitreal injections in both eyes of putative stem cells prepared from his abdominal subcutaneous adipose tissue. The full procedure from harvesting of adipose tissue to intravitreal injection had a total duration of about two hours and was carried out at a private clinic in New Delhi, India. Further details about the procedure were not known.\nThe patient was observed and demonstrated a stable condition for several months (). Eight months after stem cell injection, we performed vitrectomy in the left eye and—after a further 10 months—also in the right eye. The dense opacities were purely localized to the vitreous bodies and cleared completely with vitrectomy ( and video, supplementary material ()). The hyaloids were very adherent to the retinae which probably was the cause of the intraoperative complications: in the left eye a localized retinal detachment was induced and in the right a retinal tear was induced, both were treated accordingly. Specimens from both eyes were examined microscopically and included few vimentin-positive cells compatible with cells of mesenchymal origin. We did not identify cells differentiated into a retinal or neuronal phenotype. Following the immediate postoperative period, no intraocular reaction was observed in either eye. Visual acuity remained poor. Epiretinal remnants from the adherent hyaloid were noted in the right eye, and these remained unchanged for several months (). |
A 57-year-old man was referred to a hospital because of progressive general malaise for a month. His medical history was unremarkable and he had no history of exposure to asbestos. At physical examination, breath sounds were absent on the left lower region. A roentgenogram showed a giant tumor in the left thorax (Figure ). The heart appeared to be compressed towards the right side. He had no other chest complaints, such as cough, chest pain, and dyspnea. Computed tomography (CT) revealed a well-circumscribed homogeneous mass, which compressed the descending aorta (Figure ). The hematological and biochemical findings were normal. Bronchofiberoscopy showed stenosis of the left lower lobar bronchus from extraluminal compression. Bronchoscopic cytology revealed no abnormal findings and no evidence of bronchitis. CT-guided biopsy demonstrated fibrotic soft tissue without evidence of malignancy but the appearance of the specimen did not have enough diagnostic strength. Spirometry showed the following results: vital capacity, 2.4 L (49% of predicted); forced expiratory volume in a second, 1.7 L (42% of predicted). Results of blood gas analysis were also within normal limits. The patient was referred to our institution for surgical treatment of a suspected SFT.\nLeft posterolateral thoracotomy through the fifth and eighth intercostal spaces was performed for the resection of the tumor. We choose the fifth intercostal space as our initial Thoracotomy site. Upon entering the pleura we could easily visualize the encapsulated circumscribed gigantic tumor. The tumor was large (20 cm × 19 cm × 15 cm), extended from the thoracic aperture to the diaphragm, and caused atelectasis of the lower lobe of the left lung. An additional incision through the eighth intercostal space was made to dissect the tumor away from the diaphragm. Because the tumor had strongly attached to the lingula of the left lung, atypical wedge resection of the lingula was performed. The main vascular pedicle of the tumor was identified in the hilum of the lung. There were several small feeder vessels from the diaphragm. The tumor was fixed to the diaphragm, and we dissected it precisely either by ligation or occlusion with diathermy. The main pedicle from the hilum was ligated with nonabsorbable ties. The tumor weighed 2150 g, and appeared smooth surfaced and well-circumscribed on macroscopic examination (Figure ). Histologically, the tumor appeared to be composed of a varying proportion of spindle-shaped cells and collagen. The neoplastic cells displayed vesicular nuclei with demarcated nuclear membranes, and dispersed chromatin. Mitoses were rare, and immunoreactivity to vimentin, CD34, and Bcl2 were positive; cytokeratin was negative (Figure ). The tumor was pathologically diagnosed as benign localized fibrous tumor of the pleura. The left lung expanded completely and pulmonary function recovered to the normal level after removal of the giant tumor. The postoperative course was uneventful and the patient was discharged 12 days after the operation. |
A 10 year-old girl presented with the complaint of palpitation to a cardiologist. She had normal physical examination and laboratory tests, except tachycardia (heart rate = 130 per minute) and low TSH levels (0.005) with normal T3 (9.46) and T4 (145). She was referred to endocrinologist for possible hyperthyroidism evaluation. The thyroid gland was normal size, with no nodularity. She was diagnosed with possible thyrotoxicosis, but due to the normal physical examination, she underwent thyroid scan to rule out possible thyroiditis, which did not show any uptake in the thyroid gland, while there was an increased uptake in the right ovary (). Pelvic trans-abdominal sonography showed a heterogeneous complex solid mass of 113 × 112 × 100 mm with volume of 670 cc in the right ovary with no ascites. The patient had no complaint of abdominal pain or pelvic pain or abnormal uterine bleeding.\nShe was treated with methimazole 10 mg daily and propranolol 40 mg daily and were candidate for surgery after being euthyroid. The patient was referred to a gynecologist with the possible diagnosis of struma ovarii for further evaluation. She underwent right oophorectomy with the presumption of teratoma combined with thyroid-stimulating hormone (TSH)-suppressive therapy following treatment with I131. Total thyroidectomy was performed to permit evaluation for metastatic disease and monitoring for recurrence by thyroglobulin levels. The pathology report of the ovary mass indicated teratocarcinoma with 60% well-differentiated follicular thyroid carcinoma and 40% well differentiated follicular-variant with tumor necrosis, microscopic capsular invasion and peritumoral lymphovascular invasion, considering stage IC of PTC () and the thyroid gland did not show pathologic features of PTC.\nFurther evaluation with whole body scan with Iodine 123 (I123) showed metastasis to lymph nodes. She had high levels of thyroglobulin and received iodine therapy (150 mCi) twice. In the follow-up whole body scan, there was no trace of iodine uptake and the patient was symptom free.\nThe patient is now under treatment with levothyroxine 0.1 mg daily. Following 8 months after surgery and iodine therapy, she is totally symptom free. |
A 33-year-old woman visited our outpatient clinic with an erosive nodule on the scalp. She had been treated for juvenile dermatomyositis and had been administered immunosuppressive agents such as cyclosporine and prednisolone since she was 1 year old. On her initial visit, physical examination revealed a dome-shaped, easy-to-bleed nodule on her parietal scalp (Fig. ). In addition, a wide-spreading scarring alopecia-like lesion was observed on the whole scalp. The size of the nodule was approximately 32 mm in diameter. Skin biopsy from the nodule revealed a dermal infiltration composed of atypical keratinocytes with dyskeratotic cytoplasm (Fig. ). Around the tumor mass, a dense infiltration of lymphocytes was observed. We diagnosed this patient as having a moderately differentiated SCC on the parietal scalp and excised the tumor with a 10-mm margin. Four years after the initial excision, another red nodule developed in the left temporal region (Fig. ). Since the histological findings of the second tumor were similar to those of the first cutaneous SCC (Fig. ), we excised the tumor with a 10-mm margin. Eleven months after the second surgical treatment, a keratotic lesion was apparent around the grafted area (Fig. ), and again we excised it with a 10-mm margin. The histological findings of the third tumor were similar to those of the first cutaneous SCC, which was a widely spread SCC in situ (Fig. ). Additionally, we performed a random biopsy on the patient's scalp, and there were no atypical cells – only homogenization of the collagen bundle with carcinosis.\nSince recurrent multi-focal lesions were detected in this case, and since a previous report suggested that IL-17 plays a key role in cutaneous SCC carcinogenesis, we employed immunohistochemical staining for IL-17 as well as IL-17R in the lesional skin of each of the cutaneous SCC in our case. Immunohistochemical staining revealed that these atypical keratinocytes strongly expressed IL-17R (Fig. ), whereas normal keratinocytes in the marginal area of the tumor did not (Fig. ). In contrast, IL-17-producing cells were detected in the dermis of both the tumor area and the marginal area (Fig. ). |
A 23-year-old woman living in a rural area of Turkey presented to multiple doctors with the complaints of headache, hyperhydrosis, and abdominal pain attacks as fainting crises in 1988. She did not recall a measurement of her blood pressure during those doctor visits. After one year of intense crises, one doctor discovered attacks of hypertension, as high as 220/140 mmHg. Another doctor in Istanbul found a giant right adrenal mass in this patient. In 1989, she underwent subtotal excision of the tumor, right adrenalectomy, and right nephrectomy, but she had cardiac arrest several times during the operation and had to be resuscitated. The postoperative pathological examination showed PHEO with extensive degenerative and congestive changes in the tumor, in addition to invasion of the adrenal capsule and right kidney (tumor weight was over 1000 g). Information to the patient’s family was provided about the short life span of the patient in the postoperative period because the tumor could not be totally resected. The patient did not go to further doctor checkups as the patient and her family were waiting for her so-called inevitable death. Until 1994, antihypertensive agents were used irregularly by the patient, but then antihypertensive agents were given regularly for high blood pressure. In 1994, 3700 mBq I-131- metaiodobenzylguanidine (MIBG) treatment was given in Germany. Since then, no specific treatment was administered for PHEO. She was diagnosed with type 2 diabetes mellitus since she was 40 years old. She had a cerebrovascular accident due to hypertension at the age of 42 with sequelae of speech problems. The family history was negative for PHEO.\nShe applied to our endocrinology clinic for the regulation of her medications in July 2017. Her current medications included phenoxybenzamine 1 × 10 mg, doxazosin 1 × 4 mg, bisoprolol 1 × 5 mg, amlodipine 1 × 10 mg, metformin 2 × 1000 mg, and gliclazide mr 1 × 30 mg.\nThe following were detected as abnormal routine laboratory tests: anemia - Hb: 10.3 g/dl, creatinine - 1.31 mg/dl, HbA1c - 6.8%, LDL cholesterol - 137.5 mg/dl. The plasma free metanephrine was 1.07 nmol/L (0.08-0.51), plasma free normetanephrine was 21.70 nmol/L (0.12-1.18), plasma 3-methoxytyramine (MT) was 0.36 nmol/L (0.00-0.17), and chromogranin A level was 699.80 ng/ml (<94).\nAn abdominal computed tomography (CT) scan showed a right-sided 75 x 37 mm irregular and heterogeneous mass lesion extending inferiorly from the diaphragmatic crus level located in the right adrenal locus compatible with local recurrence (Figure , Figure ).\nThe lesion showed heterogeneous enhancement with occasional cystic necrotic areas and a calcified focus. It was observed that the mass was invasive to the vena cava inferior and showed nodular invasion approximately 1 cm in diameter in the subcapsular area in the inferior segment 6 level of the right lobe of the liver. The appearances that suggest the tumor thrombus is remarkable in the vena cava inferior. Metastasis was not detected in the patient's thorax computerized tomography scan.\nAfter a multidisciplinary discussion, an extensive surgery and then chemotherapy were recommended to the patient. The patient did not want to be reoperated on and refused to have advanced workup including genetic studies. 131-I-MIBG treatment was planned because our patient did not accept surgery and chemotherapy. For this reason, screening with I-123-MIBG was performed. Unfortunately, there was no I-123-MIBG uptake in our patient. 68Ga-DOTA(0)-Tyr(3)-octreotate (68Ga-DOTATATE) positron emission tomography (PET) /CT scanning was planned but the patient refused it. |
A 26-year-old man with a moderate extent of mental disability was admitted to the Department of Dermatology for diagnostics and treatment. He had a 2-year history of recurring painful erosions on the oral mucosa (about 3–7 times a year), recent history of rapidly evolving ulceration on the skin of the scrotum and a long history of recurring headaches and nonspecific pain in the chest. Physical examination revealed two ulcerations with necrotic bottoms on the skin of the scrotum (Fig. a), multiple aphtae on the oral mucosa (Fig. a) and one large erosion in the area of the right hip (Fig. a). Moreover, acne-like lesions were present on his face and back skin. The patient’s family history was also intriguing, since he reported an aortic aneurysm in his mother and death of unknown cause of his three brothers and mother’s sister at a young age. At the hospital, ophthalmologic and cardiologic consultations and laboratory tests revealed no significant abnormalities. Syphilis and HIV infection were also excluded. A laryngologist and dentist advised treatment of the oral aphtae and teeth. The patient underwent angio-CT of the chest and abdomen, which revealed no significant abnormalities and excluded aneurysms. Head MRI revealed small focal lesions in the white matter of the brain. Then, the examining neurologist noted only mild anisocoria but supported the suspicion of BD considering the whole clinical picture. The culture grown from the swab taken from the oral aphtae was negative, and the one taken from the genital lesions was positive for Escherichia coli. Azathioprine was introduced, first 50 mg and then 100 mg per day, along with topical treatment with silver, antiseptics and glucocorticoids as well as antibiotics (topical and systemic according to culture outcome). Great improvement of the skin lesions was achieved within only 1 month (Figs. b, b, b). Six months later, the patient experienced an episode of psychosis for which he was hospitalized at the Psychiatry Department and received zuclopenthixol and olanzapine. He has been treated in an ambulatory psychiatric care setting ever since. The patient was again admitted to the Department of Dermatology with a new erosion on the scrotum, which was successfully treated topically with glucocorticoids, and azathioprine was continued. Nine months later, the patient was again admitted to our department for a follow-up with no visible erosions or ulcerations but at that time features of Cushing syndrome were observed; therefore, the patient was referred to an endocrinologist who excluded hypercortisolemia. Three and 7 months later, the patient presented to the hospital again because of the recurrence of multiple oral aphtae. The patient is under constant and routine follow-up; he receives 100 mg per day of azathioprine and remains in good general condition with no skin involvement. He was referred to a doctor for genetic consultation. The patient provided informed written consent for the photographs and publication of this case report. |
A 60-year-old Middle Eastern male, with no significant medical history, presented to the emergency room complaining of acute nonradiating epigastric pain started about 6–7 h earlier. There was no vomiting, nausea, or anorexia. There was no jaundice, pallor, or history of loss of weight. The temperature was 37.5°C. Abdominal examination was negative for hepatomegaly, splenomegaly, abdominal tenderness, or rebounding pain. There were no signs of lymphadenopathy. A clinical diagnosis of acute gastritis was made, and the patient was given proton pump inhibitor and discharged.\nTwo days later, the pain started to radiate toward the back, and the patient started to suffer from severe thoracic back pain. Four days later, the pain started to radiate toward both lower limbs with the subtle beginning of the weakness of the lower limbs and progressed within a few hours later to inability to walk, with intact neurological function of the upper extremities.\nHe denied any history of trauma, recent history of viral illness, or illicit drug use. On neurological examination, the patient was found to have bilateral numbness and motor impairment in the lower limbs with muscle strength of 2/5. Hyperactive patellar and Achilles reflexes were observed. Pain and light touch sensation were objectively normal; there was no bowel or urinary incontinence. The straight leg raise test was negative bilaterally.\nMagnetic resonance imaging revealed a space-occupying lesion in the spinal canal at the T6/T7 level without detection of any lesions elsewhere []. Because of suspected metastasized tumor, further investigations were undertaken. Computed tomography (CT) scan of the neck, thoracic, abdomen, and pelvis were performed and did not reveal any masses, enlarged visceral organs, or lymph nodes. A bone marrow biopsy presented no marrow involvement.\nIn view of the rapid progression of the neurological deficit, the patient emergently underwent a neurosurgical decompression through partial laminectomy with total resection of the extradural mass.\nThe pathohistological examination of the biopsy revealed diffuse malignant infiltration of large atypical lymphoid cells, large vesicular nuclei, prominent nucleoli, and coarse chromatin.\nNumerous mitotic cells were also present []. Immune stains were positive for CD20 and leukocyte common antigen [] and negative for CD3 [] and CD30 []. These findings are consistent with diffuse large B-cell lymphoma. After the diagnosis was made, the patient underwent chemotherapy.\nDay after day, the patient gradually regained the strength in all muscle groups and was able to walk 1 month after surgery. Furthermore, the patient has been followed up for over 2 years without any signs of recurrence both clinically and radiographically []. |
A 60-year-old female presented to the emergency department in our hospital with acute bilateral eye vision loss. Her past medical history included an undocumented splenectomy, diabetes mellitus, and hypothyroidism. Two weeks prior to the onset of visual symptoms, she reported flu symptoms, including fever, myalgia, and dry cough. The patient neglected these symptoms and did not benefit from any treatment. Fifteen days later, the patient presented with a sudden drop in visual acuity in the left eye without pain, eye redness, or headache which became blinding after 24 hours with involvement of the contralateral eye after four days, associated with right hemibody weakness. Her admission parameters were as follows: pulse 94 beats per minute (bpm), blood pressure 135/88 mmHg, temperature 38.2°C, and oxygen saturations were 85% on room air.\nHer neurological examination on admission revealed complete bilateral blindness with no perception of light. Pupil responses to light were abolished with horizontal nystagmus and unremarkable fundoscopic findings. No other obvious abnormal findings in cognitive function, cranial nerves, or neck rigidity were demonstrated. Motor system examination revealed normal bulk in all four limbs. There was hypotonia, most marked on the right side. Power was grade 4/5 in the right hemibody and 5/5 in the left side. There was no involuntary movement or seizures. Plantar reflexes showed bilaterally Babinski signs. All modalities of sensation were conserved. Clinical examination of other systems revealed no abnormalities She presented with breathlessness a few hours after her admission to the neurology department. She was then transferred to the intensive care unit following clinical deterioration and continued increasing oxygen requirements where she was started on non-invasive ventilation.\nThe patient had magnetic resonance imaging (MRI) (1.5 Tesla) of the brain and orbits with and without contrast, which revealed right parietal nodular signal abnormality in the posterior limb of the internal capsule (Figures -)". No intramedullary abnormal signal or enhancement was present.\nBiochemical and cytological studies of the cerebrospinal fluid (CSF) showed an elevated protein count (1.41 g/L) and normal glucose without pleocytosis. No oligoclonal band was found in the CSF analysis. Blood and CSF cultures were negative. SARS-CoV-2 ribonucleic acid (RNA) in the CSF was not detected. She tested negative for myelin oligodendrocyte glycoprotein immunoglobulin G (MOG-IgG) and serum aquaporin-4 IgG antibodies. The serum paraneoplastic panel was assessed at the Mayo Clinic, and the serum angiotensin-converting enzyme (ACE) levels were normal. Blood tests showed a highly elevated C-reactive protein (CRP) at 300 mg/L with marked hyperleukocytosis (15,510/μl). Other laboratory results, such as copper, B12, and zinc levels, were checked and were within the reference range. Her nasopharyngeal swab reverse transcription-polymerase chain reaction (RT-PCR) was negative for SARS-CoV-2. COVID serology was positive for immunoglobulin M (IgM) and IgG antibodies. Other serological tests for syphilis, human immunodeficiency virus (HIV), human T-cell leukemia virus, types 1 and 2 (HTLV I/II), Epstein-Barr virus, cytomegalovirus, and hepatitis B and C were all negative. Serum ferritin (42.92 μ/L) and interleukin-6 levels (7.8 pg/mL) were normal. They were only measured on Day 5 of her admission (15 days after symptom onset). The patient reported no family history of cancer. Computed tomography (CT) imaging of the thorax, abdomen, and pelvis was done to assess possible malignancy, which was negative. The patient received seven sessions of plasmapheresis and motor physiotherapy sessions, with the prevention of thromboembolic complications by anticoagulant therapy. Her clinical evolution was stationary. A second control by encephalic and medullar MRI was performed and showed stability of the lesion load with disappearance of contrast enhancement with optic atrophy (Figure ).\nDuring her stay in intensive care, she benefited from an immunoglobulin cure (0.40 g/kg per day for five days) associated with corticosteroids. The evolution was marked by the remission of the respiratory discomfort, an improvement in the motor deficits on the right side (5/5 proximal, 4/5 distal), and the persistence of blindness. The patient was then discharged with regular neurology follow-up. |
In May 2016, a 67-year-old woman came primarily to our hospital for a consultation about painless mass of the left lower gingiva. Intra-oral examination showed a 46 × 25-mm tumor with induration on the left lower gingiva (Fig. ). A submucosal mass, independent of the gingival tumor, was palpable in the left buccal region. Several cervical lymph nodes on the left side were also palpable. Pathological examination of a biopsy sample taken from the gingival tumor revealed a well-differentiated squamous cell carcinoma.\nA computed tomography (CT) scan with contrast showed a large gingival tumor, with destruction of the adjacent mandibular bone, and four metastatic left-cervical lymph nodes that were markedly enlarged, non-homogeneously enhanced, and partially necrotic. These lymph nodes included two left submandibular and two left upper jugular nodes. CT imaging showed no metastases to the lungs. Magnetic resonance imaging (MRI) showed a large primary tumor on the left side, with its epicenter located in the lower gingiva. The tumor appeared to extend into the sublingual space medially and into the buccinator muscle laterally. A non-homogeneously enhanced mass was identified in the buccinator space along the facial vessels, anterior to the anterior edge of the masseter muscle, and lateral to the buccinator muscle (Fig. ). This mass lay on the cranial side of the primary tumor. The mandibular ramus and pterygoid region that are on the cranial side of BN were not invaded by primary tumor (Fig. ). Moreover, T1-weighted MRI showed a thin layer with high signal, indicative of fatty tissue, between this mass and the primary tumor, indicating that the mass was independent of the primary tumor. Based on its anatomic location, the mass appeared to be metastatic disease to BN. Greyscale sonogram showed some metastatic cervical lymph nodes on the left, and metastatic BN. These cervical lymph nodes were markedly enlarged, round in shape, heterogenous hypoechoic, and without an echogenic hilus. Metastatic BN was round in shape, hypoechoic, with well-defined borders, and without an echogenic hilus.\nThe tumor was diagnosed as a cT4aN2bM0 squamous cell carcinoma of the lower gingiva. The patient received neoadjuvant chemotherapy, consisting of docetaxel 60–70 mg/m2 and cisplatin 70 mg/m2 on day 1, and 5-fluorouracil 700 mg/m2/day 96 h continuous infusion. Gross examination after two cycles of chemotherapy showed marked shrinkage of the primary tumor. A slight reduction in BN size was observed. According to the Response Evaluation Criteria in Solid Tumors (RECIST) guidelines, version 1.1 [], this patient showed a partial response to treatment.\nThree weeks after the end of neoadjuvant chemotherapy, the patient underwent surgery, consisting of suprahyoid neck dissection (levels I–II) on the right side, classical radical neck dissection (levels I–V) on the left side, segmental mandibulectomy, and oromandibular reconstruction with a scapular osteocutaneous flap. The primary tumor and buccinator space including BN were dissected in continuity with neck dissection. Histopathological examination of the segmental mandibulectomy specimens showed that the alveolar bone and part of the bone trabeculae of the mandible had been resorbed and replaced by fibrous connective tissue. This tissue contained a few nests of squamous cell carcinoma, composed mainly of necrotic tissue with a small number of viable cancer cells and remnants of keratin pearls. The surgical margins were free from tumor. Metastatic disease was detected in five cervical lymph nodes, including one left submandibular aggregated-node, three left upper jugular nodes, and one left middle jugular node. No metastatic nodes revealed extra-nodal extension. Metastasis to BN was also present (Fig. ). These metastatic regions contained few viable cancer cells and consisted primarily of necrotic tissue.\nFollowing surgery, the patient was treated with adjuvant radiotherapy (50 Gy/25 fractions) with concurrent oral chemotherapy (S-1, 100 mg/day for 5 days per week for 5 weeks) []. Two years later, there has been no evidence of tumor recurrence or metastasis. |
A 3-month-old male was seen in our pediatric oncology department because of a growing parotid gland mass. The mass was congenital and was followed by the pediatric oncology team with a prediagnosis of hemangioma. The patient’s magnetic resonance imaging (MRI) scan showed an infantile hemangioma in the proliferative phase filling the parapharyngeal space ().\nThe child then received propranolol therapy, but despite this treatment the mass continued to grow, and the child was referred to our department at the age of 10 months. The otorhinolaryngological examination revealed an 8×7-cm mass filling the left parotid region, with normal facial nerve motor functions (). Histopathological diagnosis of the mass was provided by an incisional biopsy as sialolipoma.\nWe planned to perform total excision of the mass with a transparotid approach. The facial nerve was monitored intra-operatively, and surgery was performed under magnification using surgical loops. A standard modified Blair incision was used, flaps were raised (), then the facial nerve was identified in a standard fashion using a tragal pointer and posterior belly of the digastric muscle as landmarks.\nThe main trunk of the facial nerve was found to be unusually elongated before pes anserinus. A soft lobular mass was encountered. A tumor involving the superficial parotid lobe was first dissected, then the deep lobe of the parotid gland and portion of the tumor filling the parapharyngeal space were gently dissected under the facial nerve (). A Jackson-Pratt drain was inserted into the wound after the excision of the tumor.\nOn macroscopic examination, the mass was found to be a lipomatous specimen, 9×8×4 cm in size. The cut surface was consistent with the appearance of a lipoma. Histopathologic examination confirmed the diagnosis of a sialolipoma. The lesion was composed of mature adipose tissue mixed with acinar, ductal structures of a normal salivary gland ().\nThe postoperative period was uneventful. The patient’s facial nerve motor functions were well preserved, and he was discharged on the third postoperative day. The patient is still followed, and has no signs of recurrence in his 24th postoperative month. |
A 51-year-old man presented at our department having experienced nasal obstruction and facial numbness over the previous 3 weeks. He had a history of epistaxis, and had been treated with electrocauterization of the left nasal septum at a local clinic 1 month earlier. After that first treatment, he experienced headache and nasal obstruction for 2 weeks and visited another medical clinic. On examination, swelling of the septum was noticed and computed tomography (CT) was performed (Fig. A, white arrow). Cone beam CT revealed occupation of the nasal airway with fluid and severe swelling of the septum. The patient was treated with I & D under local anesthesia. In spite of the treatment with oral antibiotics, his symptoms did not improve, so he visited our department. On examination, there was diffuse swelling of the nasal dorsum and severe swelling of the septum (Fig. B, white arrow). The nasal cavity had no patency, so we undertook an emergency operation involving I & D under local anesthesia to treat the septal abscess.\nHe had no medical history or smoking history. On laboratory tests, white blood cell count was 8960/μL, erythrocyte sedimentation rate was 59 mm/hour, C reactive protein was 23.5 mg/L and serum cholesterol was 292 mg/dL. A left vertical hemitransfixion incision was made and 4 mL of purulent material was drained. There was no quadrangular septal cartilage. After saline irrigation, a silicon drain was inserted into the septal mucosa. Intravenous antibiotics were prescribed with 2 g of ceftriaxone every 12 hours and 0.5 g of metronidazole every 8 hours.\nOn the 5th postoperative day, the patient complained of blurred vision in his right eye. Visual acuity of the left eye was 0.5, but acuity of the right eye was finger count at 50 cm. Examination of the right eye revealed a whitish fan-shaped corneal opacity on the medial side with neovascularization, diagnostic of lipid keratopathy (Fig. C). Eye drops including moxifloxacin, prednisolone, and ketorolac were prescribed. We proposed that there was an infection or thrombus of the cavernous sinus; however, magnetic resonance imaging (MRI) showed no abnormal finding in the cavernous sinus or other vessels (Fig. D). An infectious organism or degenerative material from the nasal septal abscess may have been the source of lipid keratopathy via the ethmoid artery which originates from the ophthalmic artery. We postulated that regurgitation of material through the superior or inferior orbital fissure may have been the main cause of this serious condition.\nThe patient was concerned about the cosmetic appearance of his saddle nose caused by the septal abscess and septal necrosis (Fig. E), so reconstruction of the nasal dorsum and septum with 7th costal cartilage was performed after 8 weeks. The postoperative shape of his nose and septum was satisfactory (Fig. F), but the visual acuity and visual field defect in his right eye remained. Follow-up at 1 year was uneventful |
An 82-year-old woman, with a body weight of 57 kg, with a medical history of treated arterial hypertension, was admitted to our intensive care unit (ICU) with the diagnosis of septic shock. First physical examination found a heart rate at 124/min and blood pressure at 80/45 mmHg without response after 1-L crystalloid fluid expansion. The patient had a polypnea at 28 cycles/min without other respiratory distress sign. The abdomen was tense and painful to the palpation. First laboratory blood test showed a hyperlactatemia-related metabolic acidosis with a pH at 7.21, a PCO2 at 24 mmHg, a bicarbonate level at 14 mmoL/L, and a lactate level at 9 mmoL/L. Abdomen and pelvis computed tomography (CT) scan with injection of contrast material showed a voluminous liver abscess [, Panel A], consistent with the diagnosis of liver abscess-associated septic shock. Rapidly, the patient developed respiratory and hemodynamic failure with the need of mechanical ventilation, and continuous norepinephrine infusion up to 8 mg/h. An empiric broad-spectrum antimicrobial therapy consisting of piperacillin/tazobactam 4 g fourth a day and one injection of 8 mg/k of gentamicin (480 mg) was administrated for up to 3 weeks. The patient underwent an emergency laparotomy surgery for microbiological sampling and drainage of the liver abscess. In the early postoperative course, the patient demonstrated crepitation of the thoracic and cervical region consistent with the diagnosis of SE. A second CT scan confirmed the presence of massive SE extending from the abdomen to the head associated with a pneumoperitoneum [, Panels B to D]. Of note, SE was found neither during the first physical examination at ICU admission nor on the first CT scan. There were no arguments, for upper airway injuries or pneumothorax. Because of the pneumoperitoneum, an early surgical complication was suspected; thus, the patient underwent a second emergency laparotomy which revealed a perforated lesion of the transverse colon. Five centimeters of colon was removed, and a colostomy was carried out. Unfortunately, despite surgery, the patient passed away few days later due to multiorgan failure. |
A 21-year-old male presented to the emergency room with complaints of central abdominal pain for the past eight to nine hours and multiple episodes of vomiting. The pain was cramping, located in the central abdominal area. The pain occurred every one to two hours and each episode lasted 15-20 minutes. The pain was associated with episodes of bilious vomiting that provided partial relief. His last bowel movement was two days ago. The patient had been experiencing multiple such episodes for the past two years. No history suggestive of tuberculosis was present. The patient had no previous abdominal surgery. The patient’s blood pressure was 114/82 mm of Hg. The patient’s pulse rate was 92 beats per minute. The systemic examination was unremarkable. The upper abdomen was distended. The abdomen was soft and no tenderness was present. No free fluid was present. Exaggerated bowel sounds were heard. Rectal examination was normal. Abdominal x-rays revealed dilated jejunal loops with abnormal air-fluid levels and no air in the colon.\nThe patient was admitted with a provisional diagnosis of acute intestinal obstruction and was managed with nasogastric decompression and fluids. The pain resolved the next day as did the distension. Patient moved bowels subsequently. The nasogastric output was 800 - 1,000 cc in 24 hours with bilious contents. The patient was allowed liquids gradually and started tolerating semisolids well. Urgent contrast-enhanced computed tomography (CECT) abdomen was done and revealed the duodenojejunal (DJ) flexure to be lying on the right side and not crossing over to the left, small bowel loops in the right half of the abdomen, lack of visualisation of the caecum in the right lower abdomen (Figure ), and reversed superior mesenteric artery and vein in relation with the vein lying to the left of the artery (Figure ). A barium contrast study was done and revealed the DJ flexure was limited to the right side of the abdomen, along with the small bowel loops (Figure ).\nA diagnosis of malrotation of the gut was made, and the patient was planned for urgent surgery. Typical findings of malrotation were seen intraoperatively (Figures , ): small bowel loops predominantly on the right side of the abdomen, hiding the colon, and the DJ flexure not crossing the midline and remaining to the right of the midline. Ladd’s procedure was performed. The postoperative period was uneventful and the patient was discharged on day 7. The patient has been doing well for the last three months and is totally symptom-free. |
A 70-year-old female who had never smoked presented with shortness of breath due to a massive right-sided pleural effusion. Two and a half liters of blood-stained pleural effusion fluid were drained. Computed tomography (CT) examination revealed a right lung lower lobe tumor in addition to the right-sided pleural effusion. A right-sided closed blind pleural biopsy and a subsequent video-assisted thoracoscopic pleural biopsy both revealed thyroid transcription factor (TTF)-1 positive adenocarcinoma which harbored an insertion mutation in exon 20 of the epidermal growth factor receptor (EGFR) gene but negative for anaplastic lymphoma kinase (ALK) translocation by fluorescent in-situ hybridization (FISH). Talc pleurodesis was performed at video-assisted thoracoscopic surgery. She was treated with erlotinib 150 mg once daily for a month without any symptomatic or radiological improvement. She then received four cycles of carboplatin (area under the concentration-time curve of 6) + pemetrexed (500 mg/m2) + bevacizumab (15 mg/kg) every 3 weeks with stable disease as the best response. Serial three monthly CT scan examinations revealed gradually enlarging right lower lobe tumor and right upper lobe nodules. When she developed symptomatic disease progression with the third serial CT scan (Fig. a), she was treated with six cycles of single agent docetaxel once every 3 weeks with stable disease as the best response. Serial three monthly chest CT scans showed stable right intrathoracic disease but multiple enlarging right axillary lymph nodes. One month after the sixth cycle of docetaxel, she developed painless erythematous skin nodules over her right breast and the lower right-sided anterior chest wall which extended across the midline to the medial aspect of the left breast with a diffuse non-tender hard right breast lump and right nipple retraction (Fig. ). Her left breast was normal on palpation. Breast ultrasound and mammogram examinations were not performed. A core needle biopsy of the breast lump showed TTF-1 positive adenocarcinoma which was negative for estrogen receptor, progesterone receptor, and human epidermal growth factor receptor type 2 and again negative for ALK translocation by FISH. She declined further palliative chemotherapy and CT scan examination then revealed stable intrathoracic disease, enlarged right axillary lymph nodes, intact fat planes between the chest wall and breast tissue (Fig. b) but evidence of omental metastases with ascites. Radionuclide bone scan at that time showed thoracic spine as well as multiple rib metastases. She was provided best supportive care and succumbed to the disease 2 months later. |
A 40-year-old man, who had undergone LT combined with partial resection of the small-bowel mesentery and transverse colon for HCC 15 months before, complained of back pain for 1 month. The patient underwent 5 cycles of chemotherapy within 7 months after LT and resection of the right abdominal wall metastasis 9 months after LT. A solitary bone metastasis was found by MRI and PET (Fig. ) in T11 vertebra 15 months after LT, which did not cause pathological fracture or spinal cord compression. No new metastatic tumors were found in the previously resected mesentery, colon and right abdominal wall, and no metastasis to the grafted liver or other major internal organs were found, though the T11 lesion was found, indicating progressive disease after chemotherapy. The patient's Tomita score and modified Tokuhashi score for evaluating the prognosis of patients with spinal metastatic tumors were 5 and 10 points, respectively.[ The patient underwent an En Bloc spondylectomy of T11 vertebra metastases using a posterior approach. The pedicle screws were inserted into both sides of the T9, T10, T12, and L1 vertebrae through the pedicle, and then the titanium alloy rods were fixed on the screws. Reconstruction was achieved by using a artificial vertebral body at the original T11 position after the T11 En Bloc spondylectomy (Fig. ). No complications occurred. A histological analysis of the T11 vertebra after surgery revealed HCC metastasis (moderate-poorly differentiated HCC).\nAfter T11 surgery, his AFP levels decreased markedly. However, his AFP levels increased rapidly again 3 months after T11 surgery, and MRI imaging indicated metastasis at the grafted liver and the greater omentum. Numerous lung metastases were discovered by MRI (Fig. ) 5 months after en bloc spondylectomy of T11. The patient was performed 5 cycles of chemotherapy, 3 times of iodine [131I] rituximab infusion and 3 times of TACE after T11 surgery. Radiotherapy was performed for multiple lung metastases to alleviate clinical symptoms. After radiotherapy, the pain symptoms of the patient were significantly improved, followed by numerous bone metastases thereafter, but no tumor recurrence occurred at the T11 surgical site. Radiotherapy was also used to treat multiple bone metastases. He ultimately died of recurrence in the grafted liver and metastasis in the lung 29 months after LT. |
The case was a 15-month-old infant girl weighing 10 kg who was referred to Vali-e-Asr Hospital, Birjand, Iran, as a result of severe respiratory distress and seizure. At the time of visiting the residency hospital, the infant had an apnea and a heart attack, for which a cardiac massage was performed and the patient was referred to Birjand city due to an aspiration pneumonia and seizure. Physical examination of the height and weight indicated normal development and head circumference.\nAccording to the statements of the patient's companion, the infant had suffered from seizures several times, one of which occurred at the emergency room and was characterized by cyanosis in the face and lips. Considering the examinations performed in the emergency department showing 50% O2 saturation without oxygen therapy, reduced consciousness in the patient, and the information from history, the patient was diagnosed with seizure and was accordingly transferred to pediatric intensive care unit (PICU) with cardiac monitoring.\nAfter a few hours of monitoring, the patient suffered repeated VT attacks (). Therefore, medication was infused within 20 minutes, including 500 mg magnesium sulfate and 10 mg lidocaine, and VT was discontinued. Furthermore, conduction of a 24-hour Holter electrocardiography (ECG) and echocardiography were put on the agenda. Contrary to expectations, after taking antiarrhythmic drugs two days after the first arrhythmia and VT, the patient again suffered arrhythmia as repeated PVC followed by VT; therefore, lidocaine and magnesium sulfate were administered. However, even despite the 20-minute infusion of these two drugs, VT did not stop. Finally, 50 mg of amiodarone was administered in 30 minutes with a D2 long check before and after the infusion.\nAfter a 24-hour Holter ECG monitoring, long QT intervals (QT = 490 ms) and VT were observed (). Given the indication of the occurrence of TdP in the patient, treatment with beta-blockers (propranolol) was initiated and all other anti-arrhythmic drugs were discontinued. Ultimately, arrhythmia of the patient was completely controlled. Furthermore, the patient's echocardiography showed left atrial enlargement, left ventricular enlargement, left-to-right shunt, and PDA with a mean diameter of 4 mm.\nAlso, all values were within the normal range in terms of patient-requested tests, including CBC (Complete Blood Count), natrium, potassium, calcium, magnesium, Blood Urea nitrogen (BUN), creatinine, Free T4, thyroid-stimulating hormone (TSH), and Venous Blood Gas (VBG).\nGiven the size of the PDA, surgery was subsequently proposed for the infant, and the patient underwent thoracotomy and PDA ligation whereby the patient's PDA was closed. Propranolol was discontinued and not used over the course of 6-month, one-year, and two-year periodic follow-ups, and the patient was asymptomatic. Also, a list of the drugs that contributed to the arrhythmia was given to the patient to avoid consumption. |
The patient was a 3-year 7-month-old female referred by her speech-language pathologist, presenting with oromyofascial dysfunction characterized by speech sound production errors, difficulty swallowing, open mouth breathing, and noisy breathing during sleep. With respect to sleep, there were reports of difficulties going to sleep, waking up two to three times per night to drink water, getting up to go to the bathroom, open mouth breathing while asleep (), snoring during sleep, and sweating more than usual during sleep. She experienced wheezing that was associated with asthma, which was treated with an Albuterol sulfate inhaler. There were reports of difficulty with effective chewing. In addition, the patient would eat around 50% of her meals, before refusing the rest. She experienced chronic cough and recurrent upper respiratory tract infections.\nPhysical examination () of the patient showed her to be well developed, well nourished, and to appear the stated age. The patient was alert, oriented, able to communicate, and respond appropriately to questions. During the nasal examination, the nose had no external deformity. The nasal septum was straight, and the inferior turbinates were grade 2 bilaterally. There were class 3 dental occlusion and a class 3 facial-skeletal relationship characteristic of anterior-posterior maxillary deficiency. Oropharyngeal examination showed grade 3 modified Mallampati tongue position and grade 2 tonsils. The patient was found to have a restrictive class 2 upper labial frenulum with tethering of the upper lip () and restrictive grade 4 lingual frenulum [] ().\nBased on the patient history and the physical examination, the assessment revealed that the patient had functional and structural mouth breathing due to open mouth posture and low tongue posture in the setting of restrictive labial and lingual frenulum. The risks and benefits of lingual and labial frenuloplasty to facilitate lip closure and proper tongue resting posture were discussed with the parents and included, but were not limited to pain, inflammation, bleeding, scarring, need for revision surgery, and failure for significant improvement. An informed consent document was signed by the parents.\nThe maxillary labial frenuloplasty was performed under general anesthesia. Local anesthesia was achieved by applying 1 cc of 0.25% Marcaine with 1 : 200000 epinephrine to the maxillary labial frenulum via a 27-gauge needle. Pressure was applied lateral to the frenulum to locate the point of maximum tension. The maxillary labial frenulum was incised at the base of attachment with the use of sharp scissors. A V-to-Y lip closure was performed with a 4-0 chromic suture applied in a simple interrupted fashion ().\nThe lingual frenuloplasty procedure was then performed. A 2-0 silk suture was applied to the tip of the tongue as a retraction stitch. Local anesthesia was achieved by applying 1 cc of 0.25% Marcaine with 1 : 200000 epinephrine to the lingual frenulum via a 27-gauge needle. The tongue was retracted in the anteroposterior direction extending to the roof of the mouth and maxillary central incisors. Tension was applied to the floor of the mouth to protect the floor of mouth salivary glands, as well as Wharton's duct. A hemostat was used to clamp the restrictive lingual frenulum 5 mm above the attachments of the sublingual gland duct. The fibrous band was gently excised with the use of iris scissors. The underlying myofascial fibers of the genioglossus muscle were dissected further, with blunt cotton tips and sharp iris scissors being used to release the muscle from the overlying mucosa. The dissection was continued until there was adequate improvement to the tongue range of motion such that the tongue could extend up towards the maxillary central incisors in maximal mouth opening position. Simple interrupted sutures were used to close the diamond-shaped defect into a vertical line, as a means to lengthen the ventral tongue, with 4-0 chromic suture applied in a simple interrupted fashion. In total, the tongue was released from a grade 4 restricted range of motion to a grade 1 range of motion (). All wounds were hemostatic at the completion of the procedure. The patient was gently awoken from anesthesia and taken to recovery in stable condition.\nThe patient returned to the clinic four days after the procedure. The wound sites were healing appropriately, and there were no postoperative complications observed. The patient's mother reported that within the first day of returning home, the patient's issues with chewing had improved significantly, and she was more interested in eating different foods. In addition, her appetite appeared to have increased, and the patient would complete her entire meal before asking for more food (as compared to having only eaten around 50% before treatment). By the fourth day after surgery, the patient exhibited closed-mouth nasal breathing while asleep (). There were no longer any observed events of snoring and/or noisy breathing. The mother reported that the patient had remained compliant with myofunctional and speech therapy.\nThe patient returned for a 2-month postoperative visit. During the examination at this visit, no scar tissue was observed, and the wound sites had closed. Grade 1 tongue range of motion was observed ().\nThe patient's family wrote in a letter to the clinic approximately six months after the procedure, mentioning that the patient was doing very well with no complications. In addition, the patient had been reported to have completely stopped mouth breathing and snoring while asleep. However, the patient was reported to still have occasional episodes of cold and cough, as well as one episode of asthma exacerbation. Finally, the patient was reported by her family to have made progress with myofunctional and speech therapy, but the goals of eliminating the tongue thrust, achieving proper resting posture, and improving speech sound production errors were not met due to early discontinuation of treatment. |
A healthy 28-year-old man had a high-velocity motor vehicle accident. The patient was ejected from the car and was reported to have lost consciousness. He was injury-free except for the following orthopedic conditions: right inferior and superior pubic rami fractures, right sacral bone fracture, left mid-shaft femur fracture, and right knee dislocation. Clinical assessment revealed the right knee was in a flexion and valgus position with a lateral subluxation of the patella. The presence of a medial skin furrow was considered as a sign of irreducibility. Range of motion assessment and special tests for right knee stability could not be performed due to pain. Distal neurovascular assessments showed no compromise and the skin condition was normal. Serial anklebrachial index was normal and computed tomography angiography was not required, as per the advice of the vascular surgeon. X-ray results showed posterolateral dislocation and magnetic resonance imaging (MRI) results showed multiple ligamentous injuries involving the anterior cruciate ligament (ACL), posterior cruciate ligament (PCL), lateral collateral ligament (LCL), and medial collateral ligament (MCL) (). MRI of the right knee also showed interposition of the vastus medialis; therefore, complete knee reduction was not feasible ().\nTypically, knee dislocation requires urgent reduction in the emergency room. Unfortunately, in this case, reduction was impossible. Therefore, a temporary spanning external fixator was applied until the other fractures were fixed to limit pain and increase patient mobility.\nAfter treatment of the other fractures, a complete preoperative plan was formed. The patient first underwent gravity flow diagnostic arthroscopy, followed by debridement of the ACL and PCL stumps. Drilling of ACL and PCL tunnels was performed using the all-inside technique, by passing the sutures through the tunnel to retrieve the graft later. Subsequently, a skin incision was made over the posteromedial side of the knee and the herniated vastus medialis muscle was removed using a finger for knee reduction. The grafts were passed through the tunnels to restore the anatomical positions of the ACL and PCL, and the ligaments were then tensioned in a standard manner. The PCL was tensioned at 90° of knee flexion, ensuring reproduction of the anteromedial step-off; the ACL graft was tensioned at a near-extension knee position. In the same operation, reconstruction of the extra-capsular ligaments, LCL, and MCL were performed using an open technique (). The patient tolerated the procedure very well and the knee was placed in a hinge brace locked from 0° to 90° and locked in extension during ambulation. Postoperatively, the patient started prone rehabilitation as per the PCL protocol and showed improvement within weeks. In subsequent visits (12 weeks after surgery), the patient was able to walk freely without walking aids. Physical examination based on stress tests of the ACL, PCL, MCL, and LCL showed that he achieved a good functional range of motion with good stability. |
A 27-year-old Japanese woman (165 cm, 48 kg) presented with progressive headache, vomiting, and diplopia that had begun 2 months earlier. The clinical examination revealed bilateral papilledema and left abducens nerve palsy. The lumbar CSF pressure was 37 cmH2O. We administered an adequate dose of Isosorbide and glycerol as well as analgesic agents for more than 2 months, but any medical treatment brought about no effect. Routine MRI and CT imaging results were normal (), and an MR venogram showed stenosis of the right TS (). An angiogram depicted the stenotic TS and occlusion of the left sigmoid sinus (). The flow from the left TS was found to drain into the vertebral plexus. Intravenous pressure monitoring demonstrated elevated pressure in the superior sagittal sinus (23 mmHg) and a 4 mmHg pressure gradient across the stenosis. We performed a balloon venoplasty of the sinus with a Jackal balloon catheter (6×40 mm; Kaneka Medics, Osaka, Japan) (, ). Postoperatively the patient’s symptoms remarkably improved and the lumbar CSF pressure decreased to 9 cmH2O. On MR venogram taken 1 week after the operation, the patency with small remaining stenosis was confirmed (), and the patient was discharged without any neurological symptoms.\nHowever, the patient suffered an increased severe headache again the next month. Her lumbar CSF pressure was observed to have risen to 22 cmH2O, and the papilledema was not improved. An MR venogram taken on postoperative day 42 revealed restenosis at the same TS portion (), and an angiogram also showed the recurrence of stenosis (). We placed a CarotidWall stent (10×31 mm; Boston Scientific, Natick, MA, USA) (), and it achieved complete dilatation without the requirements of any boost of balloon dilatation (). On intravenous pressure monitoring, the 23 mmHg in the superior sagittal sinus with a 6 mmHg pressure gradient across the stenosis improved to 3 mmHg without a pressure gradient after the stenting. The patient’s symptoms vanished just after the stenting and had not recurred at the 6-month follow-up. The papilledema was also remarkably improved (). |
A 45-year-old male patient of East African origin presented to our tertiary care spine institute with complains of low back pain. Patient had difficulty in walking or standing and he could barely sit erect for 15 min. Visual analog scale (VAS) score for back pain was 8. Pain was only relieved on lying down. There were no radicular symptoms. He had a previous history of disk prolapse at L5-S1 level and was operated elsewhere for the same with an open discectomy. However, post-procedure, his pain had increased to such an extent that he was unable to mobilize without support. He had severe febrile illness for 1 week followed by a low grade fever that was persistent at the time of presentation to our institute. The patient was a known case of diabetes mellitus for 3 years, medicated with a single, oral glucose-lowering agent. Patient presented to us 1 month post the first surgery.\nThe patient had midline and para-spinal tenderness over L5-S1 region. Movements of the spine were grossly restricted due to pain. The patient was afebrile. On admission, the blood investigations showed an erythrocyte sedimentation rate of 65, C-reactive protein of 34, and total leukocyte count 10700. Radiographs of the lumbosacral spine revealed lytic lesions at L5 and S1 with focal areas of endplate erosion and subchondral sclerosis which was compatible with diagnosis of PS at L5-S1 (). Magnetic resonance imaging (MRI) showed erosion of end plates with enhancing pre, para, and epidural abnormal soft tissue components and patchy enhancing marrow lesions at L5 and S1 (). The diagnosis was further confirmed on the gadolinium contrast-enhanced MRI (). Computed tomography () findings of the lumbosacral spine corroborated with the plane radiograph and MRI. Blood and urine cultures were sent to look for any other source of infection. Blood culture in our case grew S. paucimobilis (). The recovery of S. paucimobilis from specimens was accomplished by the processing of blood cultures in a Bactec Model 9240 (Becton-Dickinson) or BacT/ALERT 3D. Identification of S. paucimobilis and antibiotic sensitivity tests was done on the Vitek II automated system.\nThe patient was treated by posterior decompression and stabilization from L4 to S1 and inter body and posterolateral inter transverse bone grafting at L5-S1. Inter body fusion was accomplished by a tricortical bone graft from iliac crest on one side and polyether ether ketone (PEEK) cage filled with local bone graft from the other due to the large bony defect. Intra-operatively, disk material and bony tissue from L5-S1 disk space and vertebral bodies were sent for histopathology and culture sensitivity studies. Post-procedure course was uneventful, the patient improved symptomatically and was mobilized on 2nd post-operative day with a walker. Patient was simultaneously started on Injection Linezolid 600 mg intravenous (IV) twice a day as per the antibiotic sensitivity of the blood culture report. Cultures from the disk tissue were negative for any organism. Patient was discharged on 6th post-operative day and IV antibiotics were continued for a month, followed by oral antibiotics for 3 weeks (Tab. Linezolid 600 mg twice daily). His VAS score for back pain improved to 2. A blood culture repeated after 7 weeks did not show growth of any organism. Patient was comfortable and performing all his routine activities without support. Follow-up radiographs showed good radiological fusion without any features suggestive of further infective process (). |
A 16-year-old Asian male was brought to the Maxillofacial Department of University Hospital of Messina. He was previously admitted at the Emergency Department of another hospital after a motorbike accident and immediately transferred to our Unit of Maxillofacial Trauma with a diagnosis of Le Fort III fracture and mandibular fracture associated with active bleeding of the right buccal mucosa. He has the pulse of 72/min and blood pressure (BP) of 112/70 mmHg with a normal FAST exam and presented 9,2 gr/dL of hemoglobin (Hb). The CT scan showed a mandibular right parasymphyseal fracture associated with complex maxillary fractures in a Le Fort III pattern (). No condylar fractures were detected. Upon the admission to our department, his Glasgow Coma Scale (GCS) was 13/15. The clinical examination revealed a mobility of the midline of the mandible and maxilla with diffuse swelling and an unusual profuse bleeding from a mucosal wound in the right upper molar region. The patient was brought to the operative room and monitored. After several attempts with local measures (packing and electrocautery), the bleeding still remained with a blood loss of approximately 500 mL. A further blood examination revealed 7,6 gr/dL Hb. The patient was then transfused with 3 units of fresh frozen plasma and 3 units of erythrocyte. Clinically no signs of neck swelling were recorded. No pulsations were detected through auscultation. Fluid therapy was done with 1,000 mL of lactated ringers solution and the patient was immediately sent to Radiology Unit. The CT scan with intravenous contrast showed a focal rounded hyperdensity in the right infratemporal region. The patient was then referred for interventional radiology to perform an angiography that represents the “gold standard” to study arterial lesions. Angiography was performed with the patient under conscious sedation. The Seldinger technique was used to catheterize the right common femoral artery with a 5F groin sheath. A 5F diagnostic catheter was placed into the right external carotid artery. The angiograms confirmed the presence of a dissecting posttraumatic pseudoaneurysm of the medium-distal portion of the internal maxillary artery (). No signs of active bleeding were observed during the angiographic study. A microcatheter (EV3 Marathon) was navigated coaxially into the parent artery over a guidewire (EV3 Silver Speed) to reach the pseudoaneurysm (). A mixture of 50% of acrylic-glue (Glubran 2) and Lipiodol ultrafluid was injected up to the complete occlusion of the lesion (). The final controls documented the complete exclusion of the pathological portion of the branch with the pseudoaneurysm ().\nFive days after the endovascular procedure open surgery with RIF was done once hemoglobin value was established at 10 gr/dL. The patient was then dehospitalized ten days after with 12 r/dL of hemoglobin.\nThe six-month clinical and radiological follow-up demonstrates good functional and aesthetic outcomes (). |
A 70-year-old man with antecedent of follicular lymphoma in complete remission presented at the Timone University Hospital (Marseille, France) in 2016 for a squamous cell carcinoma of the hypopharyngeal region. The patient categorically refused any treatment, including preservative surgery, radiotherapy, chemotherapy or supportive care.\nOne year later, he was addressed to our palliative care unit by the hand-surgery department after attempting suicide. The patient explained his action by the fear of suffering. No depressive state was diagnosed by our psychiatrists. Despite persistence fear of suffering, the patient rejected the idea of suicide because of his family, but still wanted to die and asks for assistance. Information on Claeys-Leonetti law was given, especially on assisted-suicide banishment and on the possibility to relieve suffering with adapted treatments.\nOne week after discharge, the patient was readmitted to our department for dyspnea and anxiety. Symptoms were managed by appropriate treatments (oxygen and low dose of midazolam in an anxiolytic purpose). Despite stabilisation, the patient was afraid of dying suffocated and asked for deep and continuous palliative sedation until death. Apart from the fear he expresses, the patient has no symptoms of anxiety, depression or pain after the introduction of appropriate treatments. On the other hand, he clearly states that he refuses to live again knowing that his death is approaching and that he is apprehensive of suffering. He says he wants to rush his death. For us, this is a request for assisted-suicide (active help from a third party for the administration of a lethal product) or euthanasia (act of a third party which intentionally provokes the death of another to put an end to his sufferings), rather than a real demand for deep and continuous sedation. It seems important to note that patient’s requests for deep and continuous sedation until death are not registered officially. The law does not even impose a written request. Thus, the request is most often made orally in the presence of several doctors and clinicians.\nIn order to try to objectify this request and therefore our answer, the patient’s request was examined and denied by palliative multidisciplinary board, in accordance with by the French Oncology Coordination Centre guidelines. This situation did not fulfil the criteria requested by Claeys-Leonetti law. Indeed, prognosis appeared not short term committed (no visible clinical progression of the disease, which commits for sure the short-term vital prognosis), symptoms were managed with appropriate treatments and no life-sustaining treatment arrest could lead to potential unbearable sufferings. Regarding the short-term criterion of life-threatening prognosis, the patient was offered to have a Computed Tomography (CT) scan to measure the progression of the disease. Indeed, no imaging had been performed for one year (time of diagnosis of recurrence). The patient refuses this proposal. The request for deep and continuous sedation was reiterated several times by the patient, who was still refusing any investigations to define the progression of his cancer and wanted parenteral hydration to be maintained. Daily, he questioned each caregiver about the rationale for the refusal of his request. How can the medical staff be sure that his prognosis is not short-term compromise? Why his psychological distress could not be considered as refractory? One week after refusing further investigation, the patient finally agrees to undergo a CT scan. Three days after the exam he dies peacefully, according to our team (no specific questionnaires or objective elements to judge the quality of death exists), of a not predictable respiratory distress certainly linked to the evolution of his cancer of the hypopharyngeal region without introduction of deep and continuous sedation, but with introduction of midazolam for anxiety. Opiates were not introduced because the patient was saying not being painful. The CT scan results, unknown at the time of death, reveal nothing conclusive (pulmonary metastases, but no lymph node involvement) and would have required additional analyzes. |
A 63 year old female without any previous medical history presented with an acute onset of dyspnea and facial swelling for 1 month duration. Physical examination showed that jugular venous engorgement was worse in the right decubitus position and we detected a cardiac systolic murmur (grade 3/6) and a third gallop. Tenderness on the right upper quadrant was noted and there were decreased breathing sounds in both lungs. The electrocardiography showed sinus tachycardia and a normal QRS axis. On laboratory findings, lactate dehydrogenase was remarkably elevated to 1,549 IU/L and the blood count was normal. Transthoracic echocardiography (TTE) and transesophageal echocardiography (TEE) revealed bi-atrial masses that were protruding into the ventricles with cardiac motion (). The chest computed tomography (CT) scan showed right inferior pulmonary artery thromboembolism and both pleural effusion and huge biatrial masses. The mass extended to the superior vena cava (). Cardiac CT showed huge bi-atrial masses and invasion into the atrial septal wall, myocardium and pericardium. We planned to do surgical resection and biopsy. The preoperative coronary angiography showed no stenotic lesions and there were feeding vessels from the right coronary artery and left circumflex artery to the biatrial masses (). A median sternotomy was made, but surgical resection was not done except for biopsies because the myocardium was rigid due to tumor invasion to the pericardium and myocardium. The pathologic examination showed that the tumor was CD20 (+), CD79a (+), CD3 (-) and CD5 (-), and this was consistent with a diffuse large B cell lymphoma (). Positron emission tomography-CT (PET-CT) showed a markedly increased fluorodeoxyglucose (FDG) uptake in the heart and pericardium as well as mediastinal and right supraclavicular lymph nodes invasion (). She was treated with rituximab, cyclophosphamide, adriamycin, vincristine and prednisone (R-CHOP). Follow-up TTE and TEE after the six rounds of chemotherapy revealed remnant, 1 cm sized, small, bi-atrial masses and reduced right and left heart failure ().\nHowever, follow-up PET-CT after the end of R-CHOP chemotherapy showed no FDG uptake in the heart except for FDG uptake of resection scar and a lung abscess that occurred during chemotherapy (). After 1st line chemotherapy, she was followed up on out patient department. After therapy for a lung abscess, we plan to try 2nd line chemotherapy. |
A 35-year-old pregnant woman at 36 weeks of gestation with a 23 × 11-mm mass and multiple lymph nodes in the left axillary up to 23 × 10-mm involvement in ultrasonography was included in this study. After performing core needle biopsy, she had been diagnosed with invasive ductal carcinoma grade II in the upper outer quadrant of her left breast. Accordingly, this tumor was characterized as negative by immunohistochemistry for estrogen and progesterone receptors, but it resulted as positive for HER2 and Ki67 positive in >25% of tumor cells. The patient at 38 weeks of gestation delivered, and then the assessment for metastasis was done. Fortunately, no metastases were found, and her cancer stage was found to be II.\nThe patient included in this study received neoadjuvant chemotherapy with both adriamycin and cyclophosphamide 4 courses. Following the breast ultrasound, clear reductions were observed in the size of the mass and axilla lymph nodes. Thereafter, the patient received paclitaxel and AryoTrust (TH) for a 12-week duration, and then AryoTrust was continued once per 3 weeks. Of note, the generic name of the trastuzumab biosimilar used for the patient was AryoTrust. Afterward, left breast quadrectomy was done, showing no tumor residue, and left axillary sentinel lymph node excisional biopsy also resulted as negative (which is a very good response to neoadjuvant chemotherapy). After performing breast-conserving surgery, radiation therapy to the whole breast and regional lymphatics were considered. This technique contains tangential fields that is performed using the 3-dimensional conformal radiation therapy. The dose provided to the whole breast was between 45 and 50 Gy over 5–6 weeks (1.8–2 Gy tumor dose daily, 5 weekly fractions), with an additional boost dose or 10–16 GY in 2 Gy fractions to the tumor bed with the patient treated with 6-MV photons.\nThe overall treatment was well tolerated by the patient. After performing 5 cycles of AryoTrust (6 mg/kg every 3 weeks), she complained of swollen eyes and urticarial lesions. By conducting the physical examination, periorbital swelling and erythema (heliotrope rash) were revealed (Fig. ). An erythematous rash on the arms (Fig. ) and chest was also observed. Also, muscle strength of the lower limbs decreased (4/5), while it was normal in all upper limbs. The laboratory examination revealed moderately elevated serum muscle enzymes (a creatine phosphokinase value: 492 IU/L with a normal laboratory range of 38–176 IU/L;lactatedehydrogenase value: 510 IU/L with a normal laboratory range of 140–280 IU/L), but fluorescent antinuclear antibody, anti-double-stranded DNA, and antibodies to histidyl tRNA synthetase were negative. Skin lesion biopsy diagnosed papillary edema, epidermal spongiosis with few eosinophilic infiltrations, and a mild interstitial mucin deposition. Since the interval between radiotherapy and the onset of the symptoms was about 6 months and a recent screening for disease recurrence resulted as negative, the observed DM symptoms were attributed to trastuzumab. For this reason, the practitioner discontinued the use of trastuzumab. With Rheumatologic consult, prednisolone and hydroxychloroquine were started. Subsequently, the patient's symptoms improved, and 1 month later, she was free of any related symptom, and CPK was found in the normal range. In the first week, AryoTrust was started with 4 mg/kg, which was then followed by 2 mg/kg weekly for 3 weeks. Afterward, the dose of AryoTrust was increased to 6 mg/kg every 3 weeks. Fortunately, the patient had no complications. |
The patient is a 13-years-old female from the Rif rural region of Morocco with a history of a non-consanguineous marriage and without any drug or disease history; she was hypotrophic and was exclusively breastfed for 6 months. Food diversification started after that with boiled vegetables, the rest was introduced starting at the age of 2 with a daily intake adapted to her age. The patient has never been operated on and has not received radiotherapy before; she also has no family history of digestive or tumor diseases. She was initially admitted to another hospital, at the age of 6 months, for the management of a staturo-ponderal delay associated with chronic diarrhea that goes back 2 months prior to her admission. The appearance was watery, sometimes fatty; the symptoms worsened by the appearance of an abdominal distension following moderate ascites. A CT scan as well as a digestive fibroscopy were done, the diagnosis of celiac disease was retained. The patient was put on a gluten-free diet, stopped after 18 months because of non-improvement and persistent diarrhea. Other clinical symptoms appeared; particularly, lymphoedema of the left upper limb at the age of one year causing the lower limb deformation and a staturo-weight delay to become more pronounced. After 3 years, the patient was lost to follow-up due to financial issues.\nAt the age of 11 years, she was admitted for the first time to our department with oedemato-ascitic syndrome made of very severe abdominal distention, fluid pleural effusion and white soft pitting edema of the lower limbs. The clinical examination found: an asthenic girl with no facial dysmorphism or mental retardation, an estimated staturo-ponderal delay of -4 standard deviation (SD) for height and −3 SD for weight, an ascites of high abundance with an umbilical perimeter at 100 cm. Lymphedema of the left upper limb with a sausage finger aspect and a dorsal face of the hand rounded into a watch glass was found as well. The patient was apyretic and the urinary strip was negative; the osteoarticular examination found a that dates back to the age of 3 years, with an internal bimalleolar distance of 35 cm as well as a small difference in the length of the two lower limbs. Recurrent warts type molluscum contagiosum in the labial and perioral region and the fingers of both hands have also been found ().\nThe diagnosis of exudative enteropathy such as Waldmann's disease was suspected in the presence of this clinical picture and further investigations were carried out. The malabsorption tests showed microcytic hypochromic anemia with a hemoglobin level of 8.5 g/L, VGM 70 fL TCMH 22 pg, low ferritinemia at 3.02 mg/L, severe hypoalbuminemia at 12g/L, total protein level at 35 g/L, hypocalcemia at 58 mg/L, phosphoremia at 33 mg/L, PTH 1–84 increased to 140.7 pg/ml, vitamin D at 4.8 ng/ml, low cholesterol at 1,05 g/L with HDL at 0.21 g/L and LDL at 0.67 g/L, hypo-gamma-globulinemia at 4.8 g/L, IgG decreased to 2.789 g/L. The rest of the laboratory work-up was unremarkable. As a part of the etiological investigations, a serum alpha 1 antitrypsin level of 1.32g/L was found, a faecal alpha 1 antitrypsin level of 0.51g/L with a significantly increased faecal clearance at 224 ml/24H, which is 10 times the normal.\nThe abdominal ultrasound showed a peritoneal effusion of great abundance with a thickening of the small bowel's wall. A thoraco-abdomino-pelvic CT scan showed, in addition to abundant ascites, an inflammatory digestive thickening associated with significant sclerolipomatosis without visible lymphatic obstruction, which is part of inflammatory digestive pathology. The ascites fluid analysis showed milky-looking chylous ascites rich in triglycerides. Fibroscopy revealed congestive gastritis without vilositary atrophy, the histopathological study of duodenal biopsies was normal. A video-capsule endoscopy was proposed to the family but due to its shortage in Moroccan hospitals as well as the financial situation of the family, it has not been completed. Other biological and radiological tests were used to rule out differential diagnoses, including trans-thoracic ultrasound with no evidence of heart failure, pericarditis or cardiac vein thrombosis, which showed a normal tuberculosis assessment, normal stool copro-parasitology and negative HIV and CMV serologies. An x-ray of the lower limbs showed signs of osteomalacia (); an additional teleradiography was requested to study the lower limb deformation. Hence, the diagnosis of the primary intestinal lymphangiectasia (Waldmann's disease) was retained before all clinical and para-clinical signs, and after the elimination of differential diagnoses. The management was difficult at the beginning because of the unavailability of the diet products in Morocco, and the high cost of octreotide for a family who lives in a low-income country. A lot of foreign associations were contacted to help the family provide the treatment from abroad. The patient was initially put on a high-protein and a lifelong low-fat diet with the supplementation of medium-chain triglycerides, with supplements of calcium, iron, vitamin D and A, and some albumin infusions during hypoalbuminemia episodes with a significant oedematous syndrome. However, the evolution after 7 months of follow-up was deemed to be a failure; hence, monthly intramuscular injection of slow-release octreotide 20 mg was added, which received good returns with clinical and biological improvement (, ).\nAn electrocardiography with monitoring of blood pressure and heart rate was performed before and after the introduction of octreotide; the patient didn't show any adverse events. After 2 years of follow-up, the patient responded and tolerated the treatment well; but with intermittent accessibility to the slow-release octreotide, because it is not available in our country. Then, during the subsequent follow-up consultations, she did not have pain and subsequent abdominal ultrasounds were proved to be normal. Treatment of lymphedema was symptomatic with multilayer compression bandages. The warts were treated with topical potassium hydroxide aqueous solution and the was treated surgically. The surgical management in our patient was performed by a senior pediatric surgeon with assistance of final year residents. |
History and physical
A 67-year-old male former smoker with a history of prior occupational asbestos exposure and recurrent bronchitis presented with progressive dyspnea and thoracic pain to the point that he could not lie down in bed. A computed tomography (CT) scan of the chest was performed, which was interpreted as right-sided pneumonia with right parapneumonic effusion. He was sent to his local emergency department, where he was admitted for antibiotics and thoracentesis, the latter which demonstrated the presence of atypical mesothelial cells with inflammatory cells. He was readmitted two weeks later for progressive thoracic pain, was found to have a recurrent right-sided pleural effusion, and was managed with partial right pleurectomy with pleural biopsy, and talc pleurodesis. Right pleural pathology demonstrated atypical mesothelial proliferation at the pleural surface, without true invasion or definitive pathologic evidence of malignancy. Following surgery, he felt substantially better, such that he could sleep in the bed again, and he was able to return to his baseline activity levels. He underwent repeat chest CT five months later, which showed right pleural thickening and a small loculated pleural effusion, favored to represent a combination of calcification, pleurodesis, and atelectasis. He remained clinically well for another five months until he presented with cough and sinus congestion unrelieved by guaifenesin, dextromethorphan, and antibiotics. He underwent repeat chest CT that showed extensive mass-like pleural thickening completely encasing the right lung, with prominent involvement of the mediastinal pleura, and probable mediastinal extension into the right paratracheal and precarinal space, with pericardial effusion and probable pericardial metastases. There was no definite invasion into the right chest wall and no evidence of disease outside of the thorax.
He then established care at our institution’s mesothelioma and pleural disease multi-disciplinary program. Pathology review of the previously biopsied pleural tumor revealed that the pleural tumor cells were positive for Wilms' tumor-1 and calretinin, and negative |
We herein describe a 42-year-old woman with HCM caused by p.Phe244Leu in MYH7 who presented with an approximately 1-week history of recurrent palpitations while eating. At 40 years of age, she had visited our hospital for a consultation regarding her condition because her mother had developed a cerebral infarction caused by atrial fibrillation and HCM. The family history showed that her uncle (the brother of her mother) had severe heart disease (details unknown) and died suddenly in his early 40s. An ECG at that time showed sinus rhythm with ST-T changes suggesting left ventricular hypertrophy. Echocardiography revealed localized mild hypertrophy of the interventricular septum with no findings of left ventricular outflow tract obstruction. The patient requested genetic analysis of HCM (approved by the Bioethical Committee on Medical Research, Kanazawa University, and with written informed consent), and we detected p.Phe244Leu in MYH7 in both the patient and her mother (Figure ).\nWe then examined the patient every 3 months, although she was asymptomatic and therefore required no medication. At 42 years of age, however, she reported episodic palpitations that occurred while eating. She also had a near-syncopal feeling in the first few days of the onset of palpitations. A physical examination was unremarkable, and her baseline thyroid function was within normal limits. An ECG and echocardiographic examination revealed findings similar to those of her prior examinations, including her left atrial size. The interventricular septal wall dimension was 13 mm. A 24-hours Holter ECG recording revealed brief runs of atrial tachycardia temporally associated with her symptoms during swallowing (Figure ). The tachycardia lasted 5-29 beats, while the ventricular rate varied from 150 to 200 beats per minute. The tachycardia occurred with every episode of swallowing and then spontaneously reverted to sinus rhythm. Upper gastrointestinal endoscopy findings were normal. Cardiac magnetic resonance imaging of the chest revealed localized mild hypertrophy of the interventricular septum, but no tumors or other abnormalities, especially near the heart (Figure ). There were no findings of late gadolinium enhancement. Thus, we diagnosed the arrhythmia as SIAT.\nWe followed her tachycardia with no drug treatment for 2 weeks, and her symptoms did not improve. Thus, we prescribed verapamil (40 mg orally, three times per day) because of its safety profile. Her symptoms did not change for 2 months, but they gradually became less frequent during the next month before subsiding completely. We discontinued the verapamil and performed 24-hours Holter ECG monitoring, which revealed no SIAT. Furthermore, no SIAT was observed on Holter ECG monitoring performed at 1, 2, 3, 6, and 7 years after the first SIAT event. At the 10-year follow-up, the patient was still free from any SIAT without medication. |
An 86-year-old man presented with a headache and blurred vision. His surgical history was significant for an uneventful left sided CEA with a Vascular Patch (Synovis VG-0106N) and utilizing a Pruitt-Inahara Shunt during the procedure. This surgery was performed 2 months ago for high grade left internal carotid stenosis (90%). Patient was having no other associated symptoms and had no clinical signs of infection. Computed tomography (CT) angiography of the head and neck was then performed, revealing a 1.2 × 2.0 cm pseudoaneurysm with a small amount of surrounding thrombus within the distal left cervical internal carotid artery just proximal to the petrous portion (). The PA was found to be distal to the endarterectomized area of the carotid artery which means that the defect was likely caused by the balloon portion of the shunt. A short segment of high grade stenosis (80%) was also revealed in the postbulbar region of the proximal left internal carotid artery (). Patient was then taken to the catheterization lab for endovascular exclusion of the PA. The right groin was prepped, and a 6 French catheter was placed in the femoral artery. An internal mammary catheter was used and then selectively cannulated the internal mammary to gain access to the left carotid artery where angiography was performed. A 0.035 wire was then passed through, and a 7 French 9 cm long sheath was passed into the left common carotid artery where a repeat angiography was performed. Then a 0.014 BMW wire was passed across those lesions, and the position was confirmed. A 6 × 50 mm long covered Viabahn stent (W. L. Gore & Associates Inc., Flagstaff, AZ) was successfully deployed across the PA, but there were still the tight stenosis and 90-degree bend of the graft anastomosis of the endarterectomy site. For the residual stenosis, a 7 × 10 stent (Nitinol Acculink) was successfully deployed and expanded using a 5 Balloon to 6 Atmospheres (). After full expansion was noted, the balloon was withdrawn, and completion angiography was performed confirming complete obliteration of the PA (). Patient recovered with no postoperative neurological symptoms and had no complications upon 3-year followup. |
Case 1: Nephritis and Rash
A 54-year-old male with conjunctival BRAF wild-type melanoma metastatic to bilateral lungs and cervical lymph nodes received nivolumab and ipilimumab combination in an expanded-access program. A history of hypertension complicated by retinopathy and a history of seasonal erythematous rash were noted. Before initiation of therapy, the advanced care provider met with the patient and family for an educational session. Potential adverse effects of the regimen were reviewed, and the patient was alerted to symptoms he needed to report. An emergency contact phone number was provided. After the patient received his first dose of therapy, the advanced care provider initiated surveillance phone calls aimed at early detection of toxicities.
The patient developed grade 2 maculopapular rash after the first dose, which was managed by holding the patient’s second dose of treatment and same-day referral to dermatology for biopsy. Acute spongiotic and vesicular dermatitis with eosinophils were identified and treatment initiated with clobetasol 0.05% topical cream. Following resolution of an acute cutaneous response, he remained on a reduced dose of prophylactic clobetasol. The role of advanced care providers is essential during dermatologic evaluation and in identifying the need for timely referral to dermatology. After evaluation by dermatology service, the patient continued on close observation during clinic visits and phone surveillance calls for rebound signs and symptoms of rash while on topical steroid treatment.
Before the start of cycle 3, the patient’s metoprolol regimen (100 mg daily) for treatment of hypertension was changed to hydrochlorothiazide at 100/25 mg daily, which resulted in mild improvement in hypertension. Following dose 3, grade 2 asymptomatic creatinine elevation occurred (1.4 mg/dL) and was managed by an increase in oral hydration. As the creatinine level increased to 2.1 mg/dL 6 days later, the patient was instructed to begin oral prednisone at 60 mg daily and discontinue the angiotensin-receptor blocker. The patient was also referred |
A 63-year-old female was admitted to the neuropsychiatric ward for ambulatory electroencephalography (EEG) to be assessed for temporal lobe epilepsy. Two months prior to admission, she had undergone another EEG that did not show electrographic seizures. The patient reported that she had been experiencing seizures since the age of 10 years. She described the seizures as a déjàvu and dissociative feeling that was accompanied by olfactory hallucinations and smelling metal. In addition, she reported prodromal symptoms such as nausea, sweating, palpitations, blank stares, greying of vision, and feeling distant. The patient also reported a postseizure state of confusion that lasted for about 1 minute. She denied experiencing any lip-smacking or incontinence. Although the patient had been experiencing seizures her entire life, she had only begun seeking treatment approximately 5 years prior. She was taking valproic acid but had been recently switched to levetiracetam due to the side effects of valproic acid. Other than seizures, the patient denied any significant past medical history. The patient reported being sexually assaulted at the age of 7 and again in her late twenties. However, the exact ages at which these events occurred are uncertain since the patient was inconsistent with her recollection.\nHer mother is alive and healthy in her late 80's without any cognitive issues. Her father passed away at the age of 92 due to a medical condition that was not otherwise specified. There is a family history of essential tremor on her maternal side.\nThe patient had worked in a large firm for over 10 years in which she had become a partner as a bankruptcy trustee. She had been asked to resign from her position two years prior due to unstable memory since she began forgetting scheduled meetings and conversations she had had with coworkers and clients. The patient had completed an undergraduate degree in commerce along with a certification for becoming a trustee. She has been married to her husband for over 30 years with no children. She drinks alcohol only occasionally and does not smoke or chew tobacco. She denies any recreational drug use (i.e., marijuana, cocaine, etc.).\nThe loss of her job, as well as her memory impairment, had caused her significant anxiety and low self-esteem. She reported being unable to remember to take her medications at the scheduled time or forgetting that she had taken them, causing her to take double the dosage. She also reported forgetting the names of people she recently met or forgetting having met them. Further, she reported confusion while navigating her surroundings and that she has previously gotten lost in familiar surroundings prompting her to call her husband for help. She also reported misplacing items around the house and difficulty finding things in the house despite having lived in the same house for many years. The patient reported that she could be cued to recall previous events; however, it was not always successful. These memory issues affected her confidence in socializing since she felt that she was being judged.\nThe presented patient was a 63-year old, well-nourished, Caucasian female. She exhibited no signs of acute distress; however, she appeared slightly anxious. She was average-statured and was of slim built. She was well-groomed and wore casual clothing that was appropriate for the interview. Throughout the interview, she exhibited no difficulty in maintaining appropriate eye contact. She was alert and oriented to person and place; however, she exhibited having difficulty orienting to time; specifically, the month and date, which was consistent throughout her admission.\nShe did not exhibit any abnormal movements. Her mood was described as “good” and her affect was euphoric with full range. Her speech was normal in pressure, rate, rhythm, and volume.\nThe patient's thought processes were circumstantial and often repetitive. She also exhibited slight thought disorganization. Her thought content did not reveal any first-rank symptoms of schizophrenia or expansive cognitions; however, it was significant for ongoing low mood and anxiousness pertaining to her memory impairment. She believed that her memory was extremely poor in both verbal and nonverbal aspects. She expressed self-doubt and compared herself to her coworkers who were of the same age and were not having any difficulties with their jobs. She appeared to hold these beliefs with great intensity and rigidity.\nRegarding her nonpsychotic preoccupations, she did not exhibit any suicidal or homicidal ideations, intentions, or plans. There was no evidence of perceptual abnormalities such as auditory or visual hallucinations or any other responses to internal stimuli during the interview. She was very insightful as evidenced by her recognition of her cognitive impairments (largely related to memory) and the fact that she sought proper diagnosis and treatment. Her judgment appeared to be good in that she did not demonstrate unsafe behaviors per se and she also proved to be cooperative and engaging.\nTo assess her cognition, the Montreal Cognitive Assessment (MoCA)—version 7.2 was conducted prior to admission. She scored 25/30, losing 5 points because of delayed recall. She had difficulties with visuospatial tasks; however, she was able to complete them with cueing. In 2013, she has previously performed the MOCA tests and had scored 29/30, losing a point for visuospatial tasks. In 2014, she scored 28/30 and in 2017, she scored 25/30, again losing 5 points for delayed recall. A day before her discharge, she completed another MOCA test and scored 25/30, losing 5 points for delayed recall. At this final test, she again displayed difficulties with visuospatial tasks but was able to accurately complete them with self-cueing.\nThe patient completed an ambulatory 3-day EEG and the results were suggestive of mild underlying bitemporal structural or functional abnormality. The results showed an improvement from those of previous EEG she had undergone since there were no intermittent 5-6 Hz sharp and notched theta activity. The change to a levetiracetam regimen appeared to have led to an interim resolution of those changes.\nShe underwent routine blood tests (i.e., complete blood count with differential, liver function test, and tests to determine the levels of thyroid-stimulating hormone, electrolytes, albumin, B12, blood urea nitrogen, and creatinine) that showed results within the normal range. A magnetic resonance imaging (MRI) assessment performed in 2018 revealed minimal microvascular ischemic changes with no evidence of acute infractions or intracranial hemorrhages. The MRI findings did not indicate specific features that were the cause of the symptoms.\nThe patient had neuropsychological assessment was completed a few months prior to her hospital admission. Patient demonstrated good effort during the assessment. However, her test anxiety was high throughout the evaluation. The Wechsler Adult Intelligence Scale—Fourth Edition (WAIS-IV) was used for evaluation. Her overall intellectual functioning was uninterpretable due to a 27-point difference between her verbal (87th percentile) and visual spatial index (27th percentile). Her processing speed was lowest at 6th percentile. Language related aspects of cognitive domain, such as task of verbal reasoning, common sense judgment, verbal reasoning between words, naming tasks, tasks providing definition of words and tasks of pronouncing words were scored at 91st percentile or above by the patient. She demonstrated a perfect performance on a task of concept formation and mental flexibility. However, when she was asked an unfamiliar task with visuospatial loading, her performance was below average. Her lowest scores were on tasks of delayed recall (verbal or visual information). She also performed poorly on recognition testing on visual memory, tasks of block design and quick scanning for visual detail.\nThe assessment notes concluded stating, that the cognitive impairment present in the patient, has a neurological component present. However, her high degree of anxiety as well as low mood was periodically controlling her behavior. Patient was noted to be easily overwhelmed and become fatigued due to her high anxiety. This, led the patient to have impaired functioning with simple tasks, causing the patient to lose her confidence. |
A 21-year-old man presented to our movement disorders clinic with a 12-year history of attacks of involuntary movements of both upper limbs and upper trunk. These attacks are often triggered by sudden movements such as standing up quickly or running on the spot. The patient was conscious of experiencing a certain “feeling” before the onset of an attack. The patient was able to prevent these attacks by standing up slowly. He once had a febrile seizure when he was 2 months old and was treated in Children Hospital No. 1 in Ho Chi Minh City with an unclear diagnosis and treatment. He had no family history of similar conditions, epilepsy, seizure, or other movement disorders. These attacks were increased in frequency to >20 times a day, rendering him from continuing his education 3 years after symptom onset. His parents thought the symptoms were related to psychological disorders, so the patient did not receive proper medical care. When he was 21 years old, he was referred to a local psychiatric hospital in Ho Chi Minh City. A brain MRI and electroencephalogram were done, but no abnormal sign was found. He was diagnosed with “conversion disorder.” He was prescribed amisulpride 400 mg ½ tablet, BID; trihexyphenidyl 2 mg 1 tablet, BID; and valproate magnesium 200 mg 1 tablet, BID, for 2 months, but there was not any amelioration. He then withdrew medications for 6 months, and the symptoms unchanged.\nFinally, he came to our Parkinson's disease and Movement disorders clinic at Nguyen Tri Phuong Hospital. Careful history taking and clinical examination showed no signs of Parkinsonism, ataxia, and no focal neurologic deficits or psychiatric symptoms including delusions, hallucinations, disorientation, and emotional volatility. Therefore, PKD was suspected. However, we could not observe the attack at our clinic due to the transient and unpredictable nature of the attacks. We then instructed the patient to self-record the attack constantly at home. After observing and analyzing the patient's video, we witnessed the manifestations of the attack, including choreic movements and dystonic posture of the upper limbs, head, neck, and upper part of the trunk. The patient usually had to hold on to the nearest table to decrease the magnitude of movements. The duration of each attack was about 10–30 s. The patient's consciousness was preserved before, during, and after the attacks. There were no neurologic deficits after the attacks (see online suppl. Video ; for all online suppl. material, see ).\nNevertheless, PRRT2 gene testing was a relatively new test in Vietnam, so it took us a few months to get the test result. The next-generation sequencing test was done by using NextSeq 550 System Guide − Illumina Inc. at Medical Genetics Institute in Ho Chi Minh City, which later revealed a mutation in c.649dupC p. (Arg217fs). Therefore, PKD with PRRT2 mutation was our final diagnosis. After a few days of carbamazepine 200 mg ½ tablet, BID prescription, the patient no longer suffered from any attack. Twenty days later, he developed skin and mucous membrane eruptions. Following a dermatologist consultation, he was diagnosed with carbamazepine-induced SJS (shown in Fig. , , , ). Therefore, carbamazepine was withdrawn, and the patient's SJS skin and mucous membrane lesions made a self-recovery without specific treatment. HLA-B gene testing was done using next-generation sequencing, but the HLA-B>*1502 allele was not found. After unsuccessful trials of levetiracetam 500 mg BID and phenytoin 100 mg TID monotherapy, the patient finally responded well to oxcarbazepine 300 mg tablet, BID, with no attack. He was followed up to 4 years at our movement disorders clinic, and oxcarbazepine dose was decreased to 300 mg 1 tablet a day in the morning. During the follow-up period, the patient had no attacks, no side effects, and no psychiatric symptoms and can continue his education. He now has a normal life and is pleased with the current treatment. However, when he forgets a dose, his symptoms re-emerge. |
A physically healthy 10-year-old boy, who weighed 32 kg (just below 50 percentile) at a height of 1.34 m (just above 50 percentile), noticed sudden atraumatic left foot deformity on waking up in the morning. There was no relevant past medical history. A visit to a local doctor was arranged promptly, and he was referred to our department for further examination and treatment within 2 days.\nOn physical examination, there was slight tenderness and swelling over the dorsal aspect of the left midfoot. The left forefoot was rigid and held in an equinovarus position (). Almost all of the load was transmitted through the lateral border of the left foot. Muscle strength was intact in the left toe extensors and flexors, and there was no sensory disturbance over his left foot. Abnormal body movements, such as tremor, clonus, tics, or dystonia, were not detected. Plain radiographs showed inversion of the left forefoot (Figures and ), suggesting incongruity of the Chopart joint complex (), which was confirmed by CT (). He was then referred to the Department of Clinical Neuroscience in our hospital. Blood laboratory results were within normal limits, and magnetic resonance images of the brain revealed no organic abnormality. There was no evidence of neurological disorder on clinical examination.\nDetailed history taking revealed that the boy had been under psychological stress. He had been playing baseball but was doing so reluctantly to please his mother. He wanted to tell his mother and the baseball team coach of his wish to quit baseball but felt unable to do so. Furthermore, his parents were divorced, and his father had remarried and started a second family. Considering all the events in his social history, we concluded his foot deformity was psychogenic equinovarus accompanied by CD. Therefore, we opted for conservative treatment rather than surgical reduction under general anesthesia.\nWhen the boy woke up on the morning after visiting our hospital, the left rigid equinovarus deformity had disappeared. However, it returned 2 days later, and then resolved completely after another 2 days. Subsequently, the clinical course was uneventful, with no return of the fixed deformity after he was allowed to quit playing baseball. He continues to have no pain or discomfort in his left foot and has no limitations on physical activity. |
A 74-year-old man, in 2013, asymptomatic right ICA stenosis was found and followed up, but in June 2017, progressive stenosis, transient left limb numbness, and an abnormal right visual field appeared. He had a previous history of brachytherapy and tumor excision for oral floor cancer in 2011 and 2014. He also had dyslipidemia, hyperuricemia, and smoking history. He was given aspirin 100 mg and bezafibrate 400 mg daily before and after the surgery. Magnetic resonance imaging (MRI) showed an old deep white matter infarct, but no new lesion was noted, and the right ICA-middle cerebral artery (MCA) was poorly visualized on magnetic resonance angiography (MRA). Single-photon emission computed tomography (SPECT) showed no apparent decrease in blood flow. Cerebrovascular angiograms showed that the right CCA was occluded immediately after its origin and blood was supplied from the right vertebral artery to the ICA through the external carotid artery (ECA) [].\nConsidering the symptomatic CCA occlusion presenting with transient ischemic attack, CEA was selected as a treatment that could achieve revascularization and plaque removal at the same time. In addition, from the results of cerebral angiography, the occluded site extended to the CCA origin, and the proximal end of the thrombus was secured using a partial sternotomy. Under general anesthesia with transnasal intubation, surgery was started under intraoperative electroencephalogram monitoring. The skin incision along the front edge of the sternocleidomastoid muscle (SCM) was extended to the front chest, and the sternum was cut into an L shape from the median cervical incision to the right second intercostal space, which exposed the CCA origin. The treatment of the precordial region was performed jointly with a cardiovascular surgeon. The proximal end of the plaque was confirmed directly under intraoperative echo and indocyanine green (ICG) video-angiography and blood flow from the ECA to the ICA through the bifurcation was confirmed on ICG video-angiography. The ICA, ECA, and CCA were clamped at sites where no blood clots had occurred, an arteriotomy was placed from the CCA to the ICA, and a shunt tube was inserted. When the lumen was exposed, a long plaque from the beginning of the CCA to the ICA was observed, which was excised in one mass. A hemashield was used to repair the CCA-ICA because primary suture was considered to have a risk of postoperative stenosis. Blood flow was confirmed by Doppler echo and the sternum was fixed with a wire [].\nOn magnetic resonance diffusion-weighted imaging after surgery, no new infarction appeared, and visualization of the main artery was improved on MRA. Computed tomography angiography confirmed good patency of the CCA. Pathological findings showed fibrous thickening and calcification of the blood vessel wall near the carotid bulb, and the lumen was narrowed. The elastic fibers in the vascular lumen were torn and ulceration was observed. Red thrombus was observed near the bifurcation, but on the central side, white thrombus rich in fiber formation was observed [].\nThere were no complications associated with the sternal incision and the patient was discharged on postoperative day (POD) 11 with a modified Rankin Scale (mRS) score of 0. There was no new cerebral infarction during the 18 months of follow-up after surgery and no CCA restenosis was observed. |
A 41-year-old man was assigned in our department with a 2 months history of stereotypic vertigo attacks. The symptoms appeared immediately after a head concussion. The initial CT scan of the head at the emergency department was without pathological findings. Due to persistent symptoms, a cranial MRI scan of the head was performed, also with normal findings. The attacks were triggered by a specific change in head position, namely when lying down into a supine position, or when looking upwards by reclining his head in sitting or standing position. He reported having a spinning sensation and a severely blurred vision during the attacks and an increasing feeling of nausea with repeated attacks. The patient was otherwise in full health and worked in a bakery. Due to vertigo, his general practitioner put him on permanent sick leave after the accident.\nApart from positioning maneuvers, the clinical neurotological, extended neurological, and ENT examination showed normal findings throughout. The video head impulse tests for all 6 semicircular canals showed normal gain values (>0.8 for horizontal and >0.7 for LARP and RALP planes) and no suspicious catch-up saccade patterns. The dynamic visual acuity test was also normal to both sides. Cervical vestibular evoked myogenic potentials showed a normal, symmetric reflex threshold of 90 dB SPL bilaterally. Ocular cervical vestibular evoked myogenic potential showed normal reflexes bilaterally with a 27% asymmetry ratio to the left side, which was interpreted as normal (cutoff 35%). Normal cyclorotation of the eyes was found in fundus photography. Video nystagmography showed normal ocular motion in smooth pursuit and saccade testing, absent head-shaking nystagmus, and a strong reaction to caloric ear irrigation on both sides without pathological asymmetry. The testing of the subjective visual vertical showed a slight tilt (2.2°) toward the left side (normal range <1.9°). Pure tone audiometry was normal and did not suggest any damage due to contusion labyrinth.\nThe Hallpike maneuver to the right side and in the sagittal plane triggered a violent down-beating nystagmus without torsional component after a latency of about 2 s, which decreased and faded out after about 15 s. At left and right gaze during the attack, the nystagmus remained purely vertical in an eye-fixed reference frame, with no additional torsional component. This pattern fits the geometric requirements for the (otolith mediated) vertical translational VOR. Interestingly, the velocity of the maneuver did not affect the intensity of the nystagmus. Even when reclining head and body very slowly, the strong nystagmus occurred after reaching about 100° head reclination (0°: upright; 90°: supine) sitting up from the head-hanging position did not trigger an upbeat nystagmus, independent the velocity of the procedure. To determine, whether pressure or circulation of ICF and blood was of influence, a slow head reclination to the left Hallpike position was performed, which triggered no nystagmus. Then the patient's legs were then lifted up (Trendelenburg's maneuver) to increase venous pressure. After a few seconds, the described nystagmus occurred.\nFirst, we suspected an anterior canal BPPV, which is often described with predominantly down-beating nystagmus in the literature []. Accordingly, we performed multiple liberation procedures for the anterior canals such as the Yakovino maneuver [] and 360° forward somersault maneuvers, as well as classical and extended maneuvers for all canals in several multi-axis turntable sessions. Overall, neither the quality of the nystagmus nor the positions in which the nystagmus was triggered could be altered. With these findings, a pressure-triggered, position-dependent cause was suspected, such as a possible third window of the labyrinth. However, other signs such as Tulio phenomenon or pressure-induced nystagmus, autophony, and hyperacusis were lacking. A new high-resolution CT of the temporal bone revealed a bony dehiscence of the right posterior SCC. Computed reconstruction in the corresponding plane showed that in supine and right Hallpike position, the dehiscence came to place at the lowest point of the SCC, possibly rendering it most susceptible to alteration of pressure and blood circulation. A symptomatic posterior semicircular canal dehiscence was postulated, and after thorough evaluation, the patient wished to take the chance of a surgical occlusion. A plugging and covering of the dehiscence in a transmastoidal, posterior fossa approach was then performed without complications and full hearing preservation. Postoperative video head impulse test showed an isolated right posterior SCC function loss as a sign of successful occlusion. Unfortunately, the described symptoms and clinical findings persisted. A new MRI again confirmed normal neurocranial findings.\nTo better understand the possible origin of the patient's symptoms, we conducted a positional mapping on the motorized multi-axis turntable, which allowed whole-body rotations relative to gravity (see online suppl. , Supplemental Digital Content; for all online suppl. material, see , which shows an example measurement). The aim was to locate the exact positions and paths, by which the nystagmus and vertigo were triggered. Eye movements were recorded by video nystagmography (EyeSeeCam, Interacoustics). The center of the head was positioned at the center of the turntable's rotation axes. Two-dimensional Helmholtz coordinates of head positions at which vertigo and DBN occurred were identified (y-axis: horizontal, space-fixed; z-axis: vertical, head-fixed; x-axis: torsional, head-fixed, unchanged). This two-dimensional area of DBN-associated head positions did not change when whole-body rotations took different paths (e.g., by forwarding pitch) or were executed with different velocities. The mapping is summarized in Figure . Approaching this area from any direction and with any velocity triggered the same nystagmus, that is, forward and backward in the tilt axis, as well as from left and right in the head-yaw axis. The quality, direction, and intensity of the nystagmus and vertigo could not be altered by any parameter. When triggered, the same nystagmus occurred every time. |
A 58-year-old man was admitted to the emergency department with right upper quadrant pain and jaundice. Though gallbladder could not be demonstrated optimally, the ultrasonographic examination revealed an increase in gallbladder wall thickness but it was contracted. Dilatation of intra and extrahepatic bile duct was detected. As a result, 64-detector multidetector computed tomography (MDCT) was performed since we could not evaluate gallbladder and choledochus optimally with ultrasound. In MDCT, gallbladder was contracted and could not be demonstrated clearly. The enlargement of choledochus and multiple choledochus stones were determined. For a better evaluation of the biliary stones, T2 weighted (T2W) MRCP (NCE-MRCP) was performed. T2W images showed multiple stones in ductus choledochus and intrahepatic bile ducts. A 15×20 mm cystic lesion including thin septa was demonstrated at the head of pancreas at periampullary region (). There was no relationship between cystic lesion and pancreatic duct. Gadoxetic-acid enhanced MRCP (CE-MRCP) was performed at the same session to examine the bile duct obstruction and to evaluate the pancreatic cystic lesion (). A slight enhancement was detected at the wall and septi of the cystic lesion in CE-MRCP. There was no relationship between cyst and choledochus or pancreatic duct. There was no obstruction in the biliary tract as well. The transition of contrast material to the lumen of gallbladder was not seen at either early or late phase images. The patient was planned to undergo the surgical treatment according to these findings.\nAt surgical exploration, the size of gallbladder was significantly reduced. There were fibrotic adhesions between gallbladder, liver and transverse colon. Cholecystectomy was done; the pancreatic cystic lesion was aspirated and excised, as well. The histopathologic examination revealed a moderately differentiated adenocarcinoma of gallbladder with positive surgical margins and the pancreatic lesion was reported as PanIN-3. The patient was referred to the medical oncology department. The patient was in good condition the 6th month after the operation. |
A 21-year-old African-American male was referred to our tertiary care cancer center with the diagnosis of unresectable, imatinib-resistant GIST. Institutional review board approval was obtained to review his clinical course. He was initially evaluated at another institution approximately one year earlier for a several-month history of gastroesophageal reflux disease, weight loss, and increasing abdominal distension. At the time of this initial presentation, the patient had these nonspecific abdominal complaints but was otherwise healthy. He underwent esophagogastroduodenoscopy (EGD), which demonstrated a large ulcerated tumor near the gastroesophageal junction. Biopsy of the mass revealed a spindle cell lesion and CD117 (i.e., c-KIT) immunohistochemistry (IHC) staining confirmed the diagnosis of GIST. Computed tomography (CT) imaging demonstrated an 11 × 10 × 8 cm lesion arising from the stomach and no other lesions elsewhere. Due to uncertainty about upfront surgical resectability, the patient was started on imatinib (400 mg/day), a tyrosine kinase inhibitor (TKI) that blocks the constitutively active receptor tyrosine kinase c-KIT, to induce adequate tumor response for potential surgical resection with negative margins [–].\nOn follow-up CT scan nearly seven months later (), there was no evidence of decreased tumor dimensions. Consequently, the dosage of imatinib was doubled to 800 mg daily. Four months after this change in dosage, the patient complained of unrelenting abdominal pain necessitating CT imaging () which revealed that the GIST had increased in size to 15 × 15 × 12 cm. This extent of disease raised new concerns for tumor invasion of the celiac axis. With failure of the downstaging approach, the patient underwent attempt at salvage surgical resection approximately one year after initial diagnosis. During diagnostic laparoscopy the tumor was considered to be infiltrative and unresectable, and a feeding jejunostomy catheter was placed for palliation. The patient subsequently sought further care at our tertiary care cancer center.\nOn our initial examination, the patient had a largely protuberant abdomen with a mass palpable from the costal margin to the pelvic brim. He was no longer able to eat and repeat EGD demonstrated a cystic/solid gastric mass arising from the level of the gastric cardia, leading to extensive extrinsic compression of the entire stomach. The celiac axis could not be visualized on endoscopic ultrasound. Pathologic review of the original biopsy specimen revealed a moderately cellular spindle cell lesion with minimal atypia and no mitosis. IHC revealed that the cells stained strongly positive for CD34 and CD117, again confirming the diagnosis of GIST. Based on our experience with GISTs, we theorized that this patient's increased extent of disease was producing mass effect and compression of surrounding organs rather than infiltrating the surrounding tissues; therefore, our surgical team recommended operative exploration.\nMore than one year following his initial diagnosis, the patient underwent resection of a massive intra-abdominal GIST with total gastrectomy and Roux-en-Y esophagojejunostomy. On our preoperative imaging the GIST measured 30 × 21 × 13 cm (), and the resected abdominal specimen revealed two masses on gross examination measuring 30 × 19 × 13 cm and 8.5 × 6 × 5.5 cm. The neoplasm showed features of high-grade GIST with mitotically active spindle and epithelioid cells involving the gastric and perigastric adipose tissue (, 60x magnification), and IHC staining demonstrated strong diffuse staining for CD117 (, 20x magnification). Despite preoperative imaging suggesting compression of the celiac axis, the mass was easily lifted off the major vessels. Unfortunately, a small isolated pelvic nodule was discovered and found to be metastatic disease, yielding a final pathologic stage of ypT4N0M1. The patient tolerated the procedure without incident and was discharged home on postoperative day 9.\nAt our institution, genetic testing of the c-KIT gene includes selective assays of exons 8, 9, 11, 13, 17, and 18. These exons are amplified by polymerase chain reaction (PCR) and then undergo Sanger sequencing. Any mutations found are then confirmed via a second independent amplification. Genotype analysis of KIT in the operative specimen revealed an exon 11 deletion in codons 557–559, which is known to confer imatinib sensitivity []. An exon 17 mutation in codon 820 of the kinase activating loop, which is associated with imatinib resistance, was also identified [–]. Given the sequencing data and presence of metastatic disease, the patient was started on sunitinib 50 mg daily given for cycles of four weeks at a time, with a two-week break between cycles. Sunitinib is a multitarget TKI against vascular endothelial growth factor receptor (VEGFR), platelet-derived growth factor receptor (PDGFR), and c-KIT that is effective in eliciting tumor response and disease stabilization in imatinib-resistant GISTs []. However, the patient progressed on sunitinib, and subsequently sorafenib 400 mg twice daily given continuously was initiated. Sorafenib is a multiple kinase inhibitor that targets VEGFR, PDGFR, RET, and c-KIT []. Sorafenib has been shown to prevent disease progression in patients with imatinib and sunitinib refractory GIST []. |
A 52-year-old woman with a history of NF1 presented with intermittent abdominal pain. She had been diagnosed as NF1 30 years ago and had a family history of NF1. Her skin showed numerous café au lait spots and multiple cutaneous neurofibromas over the whole body. A physical examination demonstrated a 20 × 15 cm palpable mass in the left abdomen. The abdominal computed tomography revealed a 15 × 9 × 7 cm tumor and several smaller masses along the jejunal loop (). On laparotomy, main tumor and numerous (more than 50) small nodular masses in the jejunum, located over 60 cm of the Treitz ligamen were found (). Segmental resection of the jejunum was performed and the patient recovered uneventfully.\nThe largest tumor was 18 × 11 × 6 cm, showed mainly extramural growth and connected to the ulcerative mass at the mucosal side. Focal hemorrhagic and necrotic portions were seen at the cut surface. Some of the multiple nodular masses, ranging in size from 0.2 to 4.0 cm, showed intraluminal umbilication. Histologically, the tumors consisted of interlacing fascicles of uniform spindle cells with eosinophilic cytoplasm and elongated nuclei (). The largest tumor had increased mitoses (19/50 high power fields) whereas the mitotic count of smaller tumors was less than 5 per 50 high power fields. Immunohistochemical staining revealed tumor cells positive for CD117, CD34 and Vimentin, and negative for S-100 and smooth muscle actin().\nGenomic DNA was extracted from each paraffin blocks of two tumors, including the extramural and intramural portions of the largest one. Exons 9, 11, 13 and 17 of KIT and exons 12 and 18 of PDGFRA were amplified using the polymerase chain reaction (PCR) and then directly sequenced. The extramural portion of the largest tumor harbored a missense point mutation (Trp557Gly) of the KIT exon 11 (). The intramural portion of the largest tumor, as well as the other tumor, had wild type KIT and PDGFRA. |
A 61-year-old male presented with a subacute history of lower thoracic back pain for few weeks. The pain was dull, nonradiating, and intermittent in nature that was not responding to analgesia. There was no history of trauma or fall. He has been diagnosed as having left choroid melanoma three years earlier. His first noticed symptoms are imbalance and left eye visual deterioration. Examination of the left funds revealed moderately large partly melanotic and partly amelanotic choroidal melanoma. The melanoma bisected the fovea of the left eye, and it had a base of 15 mm and a thickness of 4.5 mm as assessed by ultrasonography. He was screened for distant metastasis. Blood tests and imaging inform of CT scans were unremarkable at the time. He received brachytherapy using an 18 mm iodine-125 radioactive plaque, which easily covered the base of the melanoma. Subsequently, he has been monitored by an ophthalmologist regularly and a month before this presentation melanoma thickness had reduced to 2.9 mm. The vision was corrected, right eye 6/6 and left eye 6/9. On examination, apart from T8–T12 spinal tenderness, there were no neurological abnormalities detected. His initial CT scan showed a compression fracture of T10 vertebrae with associated retropulsion of the superior fracture fragment of approximately 3.5 mm and a canal stenosis at that level; findings were confirmed on MRI spine. MRI spine also showed disseminated spinal metastasis at various vertebrae without any additional spinal cord compression. He was commenced on steroids and underwent T10 laminectomy and debulking along with T8 to T12 pedicle screw fixation. He was planned for palliative radiotherapy on recovery. Histopathology from T10 vertebral body was suggestive of metastatic melanoma and immunohistochemistry showed strongly positive SOX10, MITF, HMB45, and S100 confirmed metastatic melanoma. BRAF-V600E mutation was not detected. He had an unremarkable postoperative recovery without any neurological sequelae and he was able to mobilise unaided. His full blood counts, liver function tests, renal functions, electrolytes, and PSA were unremarkable; serum lactate dehydrogenase (LDH) levels were 1716 IU/L (normal range: 105–333 IU/L) elevated. Myeloma screening was negative for paraprotein. His staging CT imaging demonstrated a right upper lobe lung spiculated lesion of size 5.6 mm × 3.8 mm × 5.1 mm that was suspicious for further metastases or a new primary lung pathology. There were suspicious lesions in liver on CT scan and were hyperechoic on ultrasound scan consistent with the hemangiomas of the liver, not appearing to have any typical features suggestive of metastatic deposits. He underwent the endobronchial ultrasound (EBUS) guided lung biopsy that was tested positive for a primary lung carcinoma; immunoperoxidase staining was positive for TTF-1, Napsin A, and Cytokeratin AE1/AE3 and there was negative staining for melanoma markers (S100, HMB45, and Melan-A) and other lung primaries (P40 and P63). Our patient was admitted again with spinal cord compression and lower limb paralysis. His general condition deteriorates quickly and collectively we aimed for palliative management as he was suffering from two advance incurable malignancies. Unfortunately, the patient died within couple of weeks. |
A 19-year old boy presented with a desire to have his eyes aligned. He complained of outward deviation of the right eye noted for 3 years, which was gradually increasing. There was no history of diurnal variation, trauma, ocular surgery, or use of glasses in the past. There was a history suggestive of recurrent vernal keratoconjunctivitis which started at the age of 5 years. He was treated with topical steroids, non-steroidal anti-inflammatory drugs, and mast cell stabilizer in the past. The patient was right handed but he was not aware of rubbing his right eye more than his left eye. At presentation, he was on mast cell stabilisers and did not complain of itching. There was no family history of atopy or deviation of eyes. The patient has one elder brother aged 24 years whose ocular examination findings were not suggestive of allergy or ectatic disorders of the cornea. On examination, uncorrected visual acuity in the right eye was 1/60 and that of the left eye was 6/36. There was absence of scissor’s reflex on retinoscopy and refraction showed high against the rule astigmatism in both eyes. Vision did not improve with refractive correction of +2DS/-12 DC at 90° in the right eye, whereas the left eye improved to 6/12 with +0.0 DS/-4.5DC at 90°. The patient had a normal head posture, and Hirschberg test revealed 15º exotropia in the right eye with full range of version and duction movements. Prism base cover test revealed a 40 prism diopter base in exotropia for near vision. The right eye was suppressed (Worth four dot test). There was mild papillary reaction in the upper tarsal conjunctiva, suggesting the presence of allergic conjunctivitis. On slit lamp examination, there was a band of thinning in the inferior cornea extending from 4 to 8 clock hours in both eyes. The band of thinning was located 2 mm above the inferior limbus. The cornea just above the thinning was steep and bulging, which could be elicited when light was shone to the limbus from the opposite side (). In these figures, a typical cone shaped bulge (like in keratoconus) was not appreciated in the light reflex. Central corneal thickness looked normal in both eyes and there was no striae, scarring, or vascularization in either eye. Munson’s sign (conical indentation of the inferior eyelid margin on down gaze, a feature of keratoconus) was absent in both eyes. Dilated fundus examination was normal. These findings were consistent with corneal ectasia in both eyes, with the right eye more affected than the left. A rigid gas permeable contact lens trial could not be done because the corneas could not hold the lens. With a trial of miniscleral contact lens, vision improved to 6/9 in both eyes. Corneal scans showed against the rule astigmatism. A crab claw pattern with central flattening of the vertical meridian was seen in the Atlas (). In the right eye, the pentacam showed that the steepest part of the cornea was just below the thinnest part of the cornea (–). In the Belin Ambrosio Display (BAD) map relatively lower BAD D values were observed, especially in the left eye (). The thinnest part of the cornea was not coinciding with the thinnest part of the epithelium in the optovue, especially in the right eye (). All of these findings suggest PMD. The right eye was more severely affected, leading to high myopia and astigmatism. This asymmetric error went unnoticed and uncorrected during his sensitive period of visual development, which led to sensory exotropia.\nThe patient was advised for follow-up for progression of PMD. If any signs of progression of PMD would be observed, collagen cross-linking (CXL) would be the treatment plan to halt further thinning and to prevent hydrops or the need of keratoplasty. For the visual rehabilitation, the patient was advised to use mini scleral lens. When the cornea stabilizes (with or without CXL), the patient will be planned for strabismus surgery. |
A 70-year-old man visited another hospital because he had experienced left chest pain for half a day. He had a history of atrial fibrillation and had taken an anticoagulant for many years. He had undergone VATS bullectomy for pneumothorax 20 years previously and the period of post-operative chest tube drainage had been long because of refractory air leakage. Plueodesis had not been performed. He had no history of hereditary diseases such as von Recklinghausen disease. Chest computed tomography showed a giant tumor in the apex of the lung and staple line of the pneumothorax surgery, however, he came home because his vital sign was stable. He returned to the hospital the next day because his left chest pain had worsened. Contrast-enhanced chest computed tomography showed left hemothorax in addition to the superior sulcus tumor and staple line of the pneumothorax surgery (Fig. a, b). The huge tumor in the apex of the lung was suspected to be an extrapleural huge hematoma, and it ruptured into the pleural cavity. He was raced to our hospital and a chest tube was inserted into the left pleural cavity, resulting in drainage of a large volume of bloody fluid. The patient then developed shock, and a massive blood transfusion was started.\nBleeding from the left subclavian artery was suspected, and emergency angiography via the right femoral artery was performed. Angiography showed some collateral arteries from the left subclavian artery to the apex of the left lung. Distal and proximal bleeding points were identified. The distal bleeding point was embolized using coils. The proximal bleeding point (Fig. a, b) was blown out, and 8.0- × 50-mm stent grafts (GORE VIABAHN; W. L. Gore & Associates, Newark, DE, USA) were placed in the left subclavian artery with careful attention to occlusion of the vertebral artery (Fig. c). The shock and bleeding continued during the angiography, and the massive blood transfusion was continued. After placement of the stents, the patient’s vital signs stabilized. The amount of bleeding was 2000 ml. The chest tube was removed 4 days after treatment, and he was discharged without sequelae. |
This paper reports a case of a 32-year-old woman presented with a 6-month history of right upper quadrant pain. The pain was controlled with oral analgesics initially, but no response off late. There was no associated jaundice, rigors or weight loss. Apart from minimal right upper quadrant tenderness, examination was unremarkable. Full blood count, liver and renal function tests and clotting profile were normal. The CA 19-9 serum level was 18 IU/l (normal 0-37 IU/l). An abdominal ultrasound scan reported thin walled 10 cm × 7.6 cm × 5.8 cm cyst in the superior part of the right lobe of liver raising a suspicion of a simple cyst of liver. There was no intra-hepatic biliary dilatation or gallstones. Computed tomography [] revealed cystic lesion in the segment VIII. The cystic lesion did not enhance postcontrast and appeared to be well circumscribed without evidence of hepatic invasion suggestive of a simple cyst. Therefore, a laparoscopic deroofing of the cyst was planned. At the time of surgery, general anaesthesia was induced and the patient was placed in a supine position. The operation was undertaken as in routine laparoscopic cholecystectomy. Port placement was initiated with a 10-mm infraumbilical trocar followed by two 5-mm ports inferior to the right costal margin along the anterior and midaxillary lines. A 10-mm port was then placed in the epigastrium. Intraoperatively [], the 10 cm × 8 cm cyst was found adherent to the superior surface of liver compressing segments VII and VIII. On further dissection, it was noted that the cyst was actually free from the liver parenchyma and was adherent to the liver by a small pedicle. Cyst fluid was aspirated without spillage. Since there was only a pedicle, it was decided to do a complete excision instead of a de-roofing. Pedicle was cauterized using bipolar and resected completely. Pathologic review of the cyst showed an inner layer of ciliated pseudo stratified mucin secreting columnar epithelium with scattered goblet cells surrounded by loose connective tissue, a discontinuous smooth muscle layer and an outer fibrous capsule these findings were consistent with a ciliated foregut cyst of hepatic origin without evidence of epithelial dysplasia or invasive malignancy. |
A 71-year-old man with progressive angina was found to have severe stenosis in the circumflex artery and complex CTO of the left anterior descending artery (, Supplementary ). Coronary artery bypass surgery was offered to the patient but declined. He underwent uncomplicated stenting of the circumflex artery, followed by staged CTO recanalization of the LAD. A guidewire was passed relatively easily across the occlusion into the true lumen of the distal LAD beyond the bifurcation. Antegrade wiring of the diagonal artery was difficult. An epicardial collateral from the distal right coronary artery was used to access the diagonal branch retrograde, and this wire was steered into the antegrade guide catheter and externalized (). Both the LAD and diagonal were dilated with 3.0 mm noncompliant balloons. The diagonal was stented into the proximal LAD, and the distal LAD was rewired. The distal LAD was dilated through the stent struts to allow passage of stents into the distal LAD (Culotte technique). The distal LAD was stented using four everolimus drug-eluting stents. The stents were postdilated with 3.0 mm noncompliant balloons in the diagonal and distal LAD and 4.0 mm noncompliant balloon in the proximal LAD. Within the distal-most stent in the distal LAD, the balloon had a persistent waist until an inflation pressure of 12 ATM. At that point, the balloon suddenly expanded. Angiography revealed contrast filling of the right ventricle (RV), with the appearance of one or two focal jets of contrast extravasation at the location of the rigid lesion in the distal LAD, consistent with iatrogenic LAD-RV fistula (, Supplementary ). Of note, the flow beyond the stents in the distal LAD was not seen, likely due to shunt flow and “coronary steal.” The patient remained hemodynamically stable and was asymptomatic, and it was elected to manage the fistula conservatively. Serial echocardiograms revealed only trace pericardial effusion. He was discharged in stable condition.\nApproximately three weeks after the CTO procedure, the patient was presented to the emergency department with chest pain and dyspnea. He described several episodes of “tearing” sensation in the chest. Blood pressure and heart rate were 123/69 mmHg and 64 beats per minute, respectively. A 12-lead ECG revealed inferior T wave inversion. Troponin I was elevated to 0.06 ng/mL. Urgent coronary angiography was performed, which revealed patent stents in the proximal LAD and diagonal branch. LAD-RV fistula appeared relatively unchanged compared to during the CTO procedure (). The right coronary artery was normal and provided a very faint collateral to the apical LAD. Left ventriculography in the left anterior oblique projection revealed no evidence of a ventricular septal defect. The patient developed profound hypotension of unclear etiology during angiography. A right heart catheterization revealed normal filling pressures, normal cardiac output and ratio of pulmonic to systemic flow (Qp:Qs) 1.7. A transthoracic echocardiogram revealed preserved ejection fraction and normal left ventricular wall motion with turbulent flow signals at the LV and RV apex throughout the cardiac cycle (Supplementary ). There was no pericardial effusion. The patient was transferred to the cardiac intensive care unit in stable condition.\nHeart team evaluation was undertaken, and it was decided to perform coil embolization of the distal LAD. A standard 6 French left coronary guide catheter was used to advance a ProGreat (Terumo Medical Corp., Somerset, New Jersey) guidewire and 2.8 French catheter into the distal LAD. This was used to deploy two Ruby (Penumbra, Inc., Alameda, California) coils at the distal end of the stent.\nThere was still persistent shunt flow, so two Tornado (Cook Medical, Bloomington, Indiana) coils were subsequently deployed. Angiogram of the LAD confirmed complete cessation of flow into the distal LAD and absence of shunt flow (, Supplementary ). Hemodynamics postprocedure demonstrated a 20 mmHg increase in systolic blood pressure and normalization of Qp:Qs. A follow-up echocardiogram revealed the obliteration of apical shunt flow, normal left ventricular ejection fraction, and no left ventricular wall motion abnormalities. Creatine kinase eight-hour postprocedure was normal. The patient was discharged home in stable condition. At follow-up, one month later, the patient remained asymptomatic. |
We report on a 73-year-old female patient with an 8-year history of thyroid nodules for which she underwent regular clinical examination and blood tests. Three weeks before referral to our endocrinological surgery unit she developed a swelling of her head and neck lymph nodes. Her past medical history included surgical removal of a benign breast tumor 22 years ago. Her family history was unremarkable regarding any endocrinological or thyroid disease. Besides her obesity she was suffering from reflux disease and hypertension. She had a regular pulse of 68/per minute and blood pressure of 120/70 mmHg under antihypertensive medication. Her muscle tone was reduced. She had observed a change of her voice and had lost 20 kg of weight within the past 4 months. A thyroid scintigram showed multiple confluencing cold nodules which were highly suspicious for malignant neoplasm of both thyroid lobes and regional lymph node metastases along both jugular veins. Clinical exam and sonography showed multiple enlarged lymph nodes of the head and neck. Pre-operative serum calcitonin was 5.3 pg/ml (normal range 0.8–9.9 pg/ml). Based on the history, clinical presentation and the scintigram we suspected an anaplastic thyroid carcinoma and performed a radical oncologic thyroidectomy with bilateral modified neck-dissection and resection of the right internal jugular vein and the left recurrent laryngeal nerve. Intraoperatively the tumor was tightly adherent to the surface of the trachea. Because of the impressing clinical picture a fine needle aspiration was not performed prior to surgery. In the authors' point of view it would have had no prognostic effect at this advanced stage of disease. A pre-operative pentagastrin test was also not performed as clinical presentation made the presence of an anaplastic carcinoma highly suspicious. Surprisingly, histopathology showed a pT3 L1 V1 R1 N1b low-differentiated medullary-thyroid carcinoma with massive lymphangiosis and haemangiosis carcinomatosa. Immunhistochemistry revealed weak positive staining for calcitonin and carcinoembryogenic antigen (CEA) (Figure ). Post-operative serum calcitonin decreased to 2.47 pg/ml still being within normal range (0.8–9.9 pg/ml). Post operatively the patient was in the intensive care unit for 6 days. A pentagastrin stimulation test was not performed due to the bad condition of the patient. A permanent tracheostoma was established to ensure a patent airway as the patient aspirated after initial extubation. Additionally massive crust formation obstructed the patient's airway which was treated with a humidifier and frequent irrigations with physiological saline solution and subsequent suctioning of the trachea. Unfortunately the patient developed central lung emboli in the right pulmonary artery. A postoperative CT scan showed multiple lung and intracerebral metastases (Figure and Figure ). The patient died six weeks after diagnosis. |
A 58-year-old lady presented with a lump of approximately 2 months' duration in left breast. Examination revealed a left breast lump of about 4 cm in the greatest dimension along with an axillary lymphadenopathy that was mobile. After a discussion, patient was operated on with left modified radical mastectomy. Histopathologic examination of the tumor revealed a disease consistent with T2 and N2. She had no metastases elsewhere (M0). Out of the 22 dissected axillary nodes, 5 were positive for malignancy. On the immunohistochemistry (IHC), the tumor was triple negative (negative for estrogen receptor, progesterone receptor, and Her2/neu). Postoperatively, she received adjuvant chemotherapy with 4 cycles of Adriamycin (60 mg/m2), and cyclophosphamide (600 mg/m2), followed by 4 cycles of paclitaxel (175 mg/m2). For the highnodal positivity consistent with N2, she was planned to have radiotherapy to the left chest wall. She received externalbeam radiotherapy (EBRT) to a dose of 50 Gy in 25 fractions starting 4 weeks after the completion of the 24-week chemotherapy course. There was no radiation recall phenomenon. Before the commencement of radiation, she had no lesions on the chest wall on the skin elsewhere.\nApproximately 9 months after the completion of radiation (the last active therapy for the cancer), the patient was found to have a depigmentation on the left chest wall congruent with area of radiation delivery. She had no pruritus in these areas. She had no previous history or family history of vitiligo or any autoimmune diseases. She had been a case of hypothyroidism with thyroxine replacement therapy. Her TSH had been in the desired range.\nThe hypopigmented lesions had clear cut margins. They coalesced and persisted in all subsequent follow-up examinations. None of the lesions were raised. They were of no definite shapes. There were no telangiectasia. Biopsy or additional testing of autoantibodies could not be performed as the patient did not consent. shows the area of hypopigmentation on the left chest wall; the photograph was obtained after 12 months of commencement of the depigmentation.\nAt the time of scribing of this report, in the last follow-up visit some 2 months previously, the patient was well and continued to have the same hypopigmented lesions on the left chest. The lesions did not appear to have regressed or progressed to contralateral chest wall or elsewhere. The patient is on periodic follow-up for the breast cancer as well as skin lesions. |
A 53-year-old female with a past medical history of morbid obesity (body mass index (BMI) of 41), osteoarthritis, a remote history of bilateral deep venous thrombosis, and severe chronic venous insufficiency (Clinical-Etiology-Anatomy-Pathophysiology (CEAP) Clinical Classification 6) presented to our clinic with refractory venous stasis ulcers of the bilateral lower extremities over the last 6 years. She was intermittently treated conservatively with PROFORE™ multi-layer compression bandaging system dressings (Smith and Nephew Inc., Andover, MA, USA) for 6-week periods with some improvement of the chronic venous stasis ulcers. A preoperative venous duplex scan demonstrated severe venous insufficiency of the superficial and deep systems of her bilateral lower extremities. Review of the preoperative duplex demonstrates an ectatic right GSV at its origin; however, a diagnosis of a venous aneurysm was not made at that time based on the images that were available ().\nThe patient was recommended to undergo bilateral lower extremity radiofrequency ablation of the GSVs to facilitate wound healing and to reduce symptoms of her chronic venous insufficiency. Endovenous ablation of the left GSV was attempted; however, a post-procedure duplex scan indicated incomplete ablation of the left GSV (). Failure of the radiofrequency ablation was attributed to the large size of the GSV (2.2 cm maximal diameter) and severe thickening of the vein from chronic inflammation. The patient was subsequently treated by open surgery with left GSV high ligation, saphenofemoral disassociation, and stripping of large varicose veins involving the left thigh and lower leg. Of note, the left GSV was diffusely enlarged, but there was no focal area of enlargement consistent with a venous aneurysm. The patient had an uneventful recovery from this surgical procedure and experienced complete healing of her left leg chronic venous stasis ulcers within a few weeks. Radiofrequency ablation of the right GSV was not performed due to factors associated with the prior failed intervention involving the left GSV. The right lower extremity ulcer was shallow, located on the medial aspect of the ankle, and measured 2.5 × 3 cm. The patient was again offered operative repair for her right lower extremity similar to the approach for her left lower extremity given the large size of the GSV and that as she had previously failed radiofrequency ablation on the left. During the high ligation of the right saphenofemoral junction, a 3 × 4 × 5 cm aneurysm was encountered (). The aneurysm was noted to be superficial to the true right deep femoral system, and the right GSV and anterior saphenous vein appeared to be directly associated with it. Repair consisted of dissociation of the GSV and anterior saphenous vein with high ligation, aneurysm resection, and stripping of large varicose veins of the thigh and lower leg. Pathology confirmed that this lesion was ectatic venous tissue consistent with a venous aneurysm. The patient recovered uneventfully and experienced complete healing of the right leg venous stasis ulcer by postoperative week 4. The patient is being maintained on compression therapy for both of her legs lifelong. |
This 54-year-old female was referred to the Neurofibromatosis outpatient clinic of the Oral and Craniomaxillofacial Surgery Department for treatment of numerous cutaneous tumors that preferentially covered her trunk and extremities. On admission, the patient described the tumors to be painless and disfiguring. Besides the cutaneous tumors the patient sought advice for treatment of a painful region of her left small finger (Figure 1 ). For some time the ulnar side of the distal phalanx of this finger had become very touch-sensitive. There was neither a tumor visible nor any other pathological alteration of the skin. However, the finger could not be examined by palpation. The suspected diagnosis was initially a glomus tumor [] or a non-palpable nodular PNF.\nMRI at 3T revealed numerous, popcorn-like, contrast-absorbing, hyperintense formations on T2-weighted images, up to 1.2 x 1.9 cm² in size located on the palmar side of the left hand (Figure 2 ). These small lesions mainly affected the metacarpal bones II and III. MRI also showed a sharply defined lesion of max. 1 cm in diameter in the subcutaneous layer at the level of the radiocarpal joint and distal to first digit that reached to the tendons of the extensor musculature. The bony structures showed a homogeneous signal. The findings were interpreted as disseminated subcutaneous and cutaneous neurofibromas of the entire left hand. Structures were found on the distal phalanx of the left small finger whose intensity pattern corresponded to that of the other lesions.\nDuring surgery for the excision and vaporization of numerous neurofibromas of the trunk and extremities, the pain sensitive finger region was also explored. After incision of the skin, no tumor was visible. Therefore, a circumscribed subcutaneous excision was taken as a tissue sample and examined histologically.\nThe tissue findings confirmed cutaneous neurofibromas for the numerous nodules excised from the trunk and extremities. Surprisingly, there was no evidence for a glomus tumor in the tissue sample of the finger. Smaller neurofibromas were differentiable in this tissue sample. Within the corium as well as in the subcutaneous adipose tissue there were some clustered Vater-Pacini (VP) corpuscles. Next to the corpuscles several small nerve fibers were present. Diagnosis of VP neuroma was made (Figure 3 ).\nThe nerve fibers were positive for S100 and neurofilament. S100 immunoreactivity was especially strong in the center of the corpuscle. The perineurium of the nerve fascicles and the lamellae of the corpuscles expressed epithelial membrane antigen (EMA).\nThe healing process of the wounds was inconspicuous. The patient noticed that the sensations of pain had diminished significantly in the area of the small finger soon after surgery. |
We present the case of a 56-year-old Caucasian man with Forestier's disease also known as diffuse idiopathic skeletal hyperostosis (DISH). Forestier's disease is a common spinal enthesopathy that is mostly encountered in men older than 50 years []. A prevalence of 28% has been found in autopsy specimens []. DISH is more common in patients with diabetes and gout [].\nOur patient sought the services of a local physical therapist while on vacation to obtain massages and other treatments for his aching and stiff neck. According to our patient, the physiotherapist (board certified per our patient's report), was more forceful in his manipulating than our patient was used to. He reports having had severe neck pain prior to a short period of unconsciousness. After the procedure, he was unable to mobilize himself off the table. Prior to this incidence the physiotherapist had reportedly not known about our patient's Forestier's disease. He supposedly had not asked about underlying diseases nor had our patient remembered to tell him.\nOur patient was referred to a local hospital with a partial tetraparetic syndrome. MRI scans of the cervical spine showed a C 6/7 luxation fracture, as well as degenerative alterations with large spondylophytes bridging the vertebral bodies of the cervical spine extensively (consistent with Forestier's disease) (Figure ). Axial traction therapy was chosen for several days followed by a dorsal stabilization procedure using internal fixation. Twelve days after the initial trauma he was repatriated and referred to our division of spine surgery. Subjectively, the symptoms improved over time after the initial trauma. On admission to our institution our patient reported having electrifying pain in the whole left upper extremity. Finger abduction in the left hand was slightly reduced to grade M4 of five (according to British Medical Research Council grading, 1978). On the right side the active finger abduction was significantly reduced to grade M0-1/5 and elbow extension to M3/5.\nA subsequent computed tomography (CT) scan showed an insufficient fracture reduction leaving the facet joints in a persistent subluxation, potentially continuing to compromise neural structures (Figure ). Our patient was also noted to have elevated inflammatory parameters. Due to his clinical presentation, revision surgery with posterior hardware removal, irrigation, debridement and decompressive laminectomy was undertaken. Our patient was then flipped into a supine position for a ventral approach. No obvious signs of infection were seen anteriorly and therefore a ventral inter-corporal fusion procedure was performed at level C6/7 and the cervical spine instrumented between the levels C5 to T1 using a plate (Vectra).\nThe results of cultures of the intra-operative biopsies taken from our patient's dorsal cervical spine were positive for a coagulase-negative Staphylococcus and Proteus mirabilis. An adequate antibiotic regime was established. Our patient was transferred to a neurological rehabilitation center eight days post-operatively in a stable condition.\nAt two-month follow-up, our patient reported satisfaction with the outcome. His inflammatory parameters had normalized and all the incisions looked well healed. He had an acceptable range of movement of his cervical spine. While his left upper extremity had full sensomotor function, on his right side function was still impaired. At six-month follow-up, persistent but slightly improving neurological deficits were recorded. Figure shows radiographic imaging results obtained at the six-month follow-up demonstrating no changes in alignment, intact hardware and osseous consolidation. Subsequent to the last follow-up, our patient was still undergoing ergotherapeutic and physiotherapeutic therapy addressing his right upper extremity limitations. |
A 63-year-old female patient was admitted to the Galactophore Department, Bao Ji Central Hospital with a huge lump in the left breast. The patient stated that they had identified a thumb-sized lump two years earlier, but that they had ignored it. From July 2011, the patient observed that the lump was growing rapidly; in February 2012 the lump had become so large that the patient’s left chest was entirely covered with a solid, irregularly-shaped, somewhat moveable mass of tremendous size and the patient was barely able to lie in the prone position. Family members then finally convinced the patient to seek medical attention. Upon physical examination, the patient presented with an elastic hard mass in the left breast that was 45 cm in diameter, with a circumference of 97 cm (). Rapid growth had resulted in shiny stretched skin that was translucent, showing underlying extremely enlarged veins with sections of the circuitous veins about to undergo ulceration, although no bleeding or discharge was noted. The lymph nodes in the axillary and other superficial areas were not palpable. The patient’s previous medical history revealed that they had high blood pressure, diabetes and atrial fibrillation. Moreover, the patient had suffered from a cerebral infarction; the patient’s right side was paralyzed and communicative disorders had appeared two years previously. The patient was unable to care for herself due to the cerebral infarction. The patient’s medical history was negative for allergies, smoking, drinking, contagions and serious addiction. Furthermore, there was no family history of breast cancer on either side of the patient’s family.\nDue to the non-compressible nature of the mass and the size of the tumor, regular breast examinations, including mammography and ultrasound studies, could not be used, while a computed tomography (CT) scan of the chest revealed a solid mass. Total body scintigraphy was negative. Comprehensive examinations and detailed assessments were additionally performed on the patient due to the presence of high blood pressure, diabetes, atrial fibrillation and cerebral infarction. In order to ensure the long-term success of the treatment, a consultation with the Respiratory Medicine, Vasculocardiology, Neurology and Anesthesiology departments was organized. Antihypertensive, glycemic control, oxygen uptake and antiarrhythmic therapies were administered prior to surgery. The pectoris major and minor had atrophied and were tightly adhered to the tumor due to long-term compression, so the surgeon decided to resect the mass along with these muscles. The mass was resected subsequent to 3 hours of surgery (). The post-operative pathology report showed an extremely large grey mass, weighing 11 kg and measuring 36×40×18 cm, which was covered by ∼32×35 cm of skin tissue. When the tumor was cut into halves along the nipple, it was observed that the majority of the tumor tissue was a myxoid, lobulated mass with multiple hollow cavities, thick, yellow, viscous fluid and certain necrotic areas. The microscopy findings showed extensive necrosis and the majority of the tumor tissue exhibited an epithelial component with a leaf-like pattern, but no cellular atypia. The final histopathological diagnosis was PT of the benign type ().\nIn view of the benign nature of this tumor, no chemotherapy or radiotherapy was administered following surgery. The patient recovered well and to date there has been no evidence of local recurrence or distant metastasis. |
A 45-year-old male, who was transferred from a local clinic, presented with a one month history of a swollen painful back. He had experienced intermittent back pain over the previous 2-year period. He denied a history of acupuncture or local injections. A mass-like swelling was palpated on the right side of his back. The range of motion of his back was slightly limited, but there was no radicular pain. The motor, sensory and deep tendon reflexes were normal in both lower extremities.\nThe white blood cell count and the erythrocyte sedimentation rate were 9,000/mm3 and 76 mm/hour, respectively. The plain radiographs indicated only subtle changes in the right transverse process of the 5th lumbar vertebra (). T2 weighted axial magnetic resonance imaging (MRI) revealed a space-occupying lesion, which was located behind the right paraspinal muscle, and it was connected to the paraspinal muscle (). Enhanced computed tomography (CT) revealed a 7 × 4 × 1 cm-sized space-occupying lesion with a rim enhancement pattern and swelling of the surrounding soft tissues. CT also revealed an osteolytic lesion (2 × 1 cm in size), which was located in the right transverse process of L5, and it was similar to Brodie's abscess in the long bones ().\nOpen drainage of the abscess and a resection of the right transverse process of L5 were performed under general endotracheal anesthesia (). Grossly, the osteolytic lesion was surrounded by a sclerotic bony margin and there was pus in the abscess cavity, and the pus had gravitated anteriorly and posteriorly to the paraspinal muscle; this suggested chronic osteomyelitis. Th e pus culture demonstrated a mixed infection with Staphylococcus aureus and coagulase-negative staphylococcus. The histological examination revealed an infiltration of chronic inflammatory cells into the dense lamellar bone spaces, which was all consistent with chronic osteomyelitis ( and ).\nThe patient received an intravenous injection of 1st generation cephalosporin (cefotiam hydrochloride 1.0 g bid) for 4 weeks, which resulted in the complete resolution of symptoms and a decrease in the erythrocyte sedimentation rate to normal and this lasted during follow-up. At the two year follow-up, the patient had normal spine functions. |
A 50-year-old man was admitted to the Hyogo Medical College Hospital for examination and treatment of an abnormal visual field in mid-July 2011. He was diagnosed with an ONB in the sinonasal cavity. The tumor extended outside of the sinonasal cavity (Kadish stage C), and chemoradiotherapy was performed. No symptoms or signs of Cushing’s syndrome were apparent at that time. Although the size of the primary lesion tended to decrease in 2012, it had extended to the opposite side of the sinonasal cavity (Kadish stage C) by 2014. By January 2015, hypokalemia, hyperglycemia, and decreased eosinophil and granulocyte counts had developed. The patient was subsequently referred to the Department of Diabetes, Endocrinology and Metabolism for further examination of the hypokalemia in mid-July 2015.\nshows changes in the serum concentrations of potassium and glucose and the blood eosinophil count during the clinical course of the disease. From 2011 to 2014, the serum concentrations of potassium and glucose and the blood eosinophil count were almost normal.\nThe patient was a social drinker who had smoked 40 cigarettes a day for 20 years prior to admission. His body weight was 67.0 kg in 2012, 86.0 kg in 2014, and 73.6 kg on admission. His blood pressure was 165/109 mmHg, and his pulse was 80 beats/min. On physical examination, a Cushingoid appearance was apparent, including moon facies, truncal obesity, purpura, and muscle atrophy of the bilateral lower extremities.\nLaboratory findings on admission are shown in . The total white blood cell count was slightly increased; the neutrophil count (both segmented neutrophils and bands) was markedly increased, while the eosinophil count was 0%. The fasting plasma glucose level was elevated. The serum concentration of potassium was low, while the serum concentration of magnesium and renal function test results were normal. Arterial blood gas analysis revealed metabolic alkalosis with respiratory compensation. Both the fractional excretion of potassium and the transtubular potassium gradient were elevated. Endocrinological examinations showed that the serum concentration of ACTH and cortisol and the urinary excretion of cortisol were markedly increased. The serum concentrations of aldosterone and dehydroepiandrosterone sulfate were normal. The urinary excretion of aldosterone and urine 17-ketosteroid was normal. However, the ACTH and cortisol circadian rhythms were deranged (data not shown). Low- and high-dose dexamethasone suppression tests were not performed because the patient’s health was poor. A pituitary tumor was not detected on magnetic resonance imaging of the brain, and tumors were not detected on a computed tomography scan of the chest. Bilateral adrenal hyperplasia was observed. Based on these physical and endocrine findings, the patient was diagnosed with EAS.\nChanges in findings of 18F-fluorodeoxyglucose (FDG) positron emission tomography-(PET) (2011, 2012, and 2015), and of octreotide PET (2015) are shown in . Two courses of chemoradiotherapy significantly reduced FDG uptake in the sinonasal cavity (PET2). However, strong uptake of FDG was again noted in the primary lesion in the sinonasal cavity, which had extended to the opposite site on PET3 by 2015, when a Cushingoid appearance had developed. The strong uptake of 68Ga-labelled 1,4,7,10-tetraazacyclododecane-N,N',N″,N0‴-tetraacetic acid-d-Phe1-Tyr3-octreotide (68Ga-DOTATOC) was also observed at the same site in 2015.\nSerial changes in the immunohistochemical reactivity against ACTH at the primary site of the ONB are shown in . All Kadish stages were stage C, and the Hyams grade of each biopsy, from Bp1 to Bp3, was grade II. The purpose of Bp1 in 2011 was to diagnose the primary lesion. Although the size of the primary lesion tended to decrease in 2012 by radiotherapy and chemotherapy, it had extended to the opposite side of the sinonasal cavity by 2014. The purpose of Bp2 was to re-evaluate the condition of the primary lesion. In accordance with the development of Cushing’s signs and symptoms in 2015, the purpose of Bp3 was to diagnose EAS. Retrospectively, all tumor cells were negative for ACTH in 2011 and 2014, while the tumor cells became positive for ACTH immunostaining in 2015. Changes in ACTH expression by the tumor cells coincided with a decreased serum potassium concentration and eosinophil count and an elevated fasting plasma glucose level.\nThe patient was treated with metyrapone (500–750 mg/day). Changes in the serum concentrations of ACTH, cortisol, potassium, and glucose after treatment are shown in . After day 20, the serum concentrations of ACTH and cortisol were markedly decreased (ACTH, 23.5 pmol/L; cortisol, 220.8 nmol/L). The patient’s hypokalemia and hyperglycemia also improved.\nWritten informed consent was obtained from the patient for publication of this report and accompanying images. The need for approval by an ethics committee or institutional review board was waived. |
The patient was a 39 year-old woman admitted to our ward because of severe abdominal pain, diarrhea and emesis for 3 days. No evidence of infectious gastroenteritis was found. She suffered from end-stage renal disease since 2007 because of aHUS due to a heterozygous disease-causing mutation resulting in a two base deletion with frameshift in the membrane cofactor protein (MCP) gene which leads to a STOP codon at AA 254. At the time she was treated with plasma exchange. In 2014 she underwent kidney transplantation. The kidney donor was a 58 year old male who died of myocardial infarction. The organ quality was considered good due to absence of any chronic alterations and achievement of primary function with a serum-creatinine of 1.2 mg/dL. In the following 2 years the patient suffered from progressively worsening transplant function which was assumed to be the consequence of a severe cytomegalovirus (CMV) infection with encephalitis and the need for nephrotoxic antiviral therapy. Unfortunately, no biopsy was performed to validate that assumption. Subsequently, in December 2018 she developed ESRD again and was treated with hemodialysis since then. The patient remained on the calcineurin inhibitor (CNI) tacrolimus as immunosuppressive therapy to avoid immunization since further kidney transplantation was planned. On admission an abdominal X-Ray showed no pathological findings, however an ultrasound suggested intussusception of the small intestine (Fig. a). Additionally, a contrast enhanced CT-scan of the abdomen showed inflammatory wall thickening of the small intestine (Fig. b). Consequently, a esophagogastroduodenoscopy (EGD) was performed, showing fresh blood in the small intestine with no clear source of bleeding (Fig. c,d).\nHistopathological findings in the small intestine showed thrombotic microangiopathy with fibrin insudations in vascular walls, endothelial swelling and fresh microthrombi leading to lumen obliteration (Fig. e,f). The biopsy from the colon was normal. Both biopsies did not show any signs of cytomegalovirus infection or any other infectious agent.\nThe histological findings were rather surprising since the patient never had signs of systemic hemolysis reflected by normal bilirubin and haptoglobin values as well as absence of fragmented erythrocytes (Table ). Solely, slightly elevated lactate dehydrogenase values could be found initially (Table ). The patient’s ADAMTS13-levels were normal. There was no systemic sign of activation of the complement system (Table ).\nSince TMA due to aHUS is often found to be triggered, we first stopped treatment with tacrolimus as a potential cause for TMA. Interestingly, during the last months the renal transplant caused pain accompanied by repeated subfebrile temperature. Therefore, inflammation due to renal infection or acute rejection was also considered as a potential trigger for TMA. Since TMA of the small intestine due to aHUS was highly probable, eculizumab treatment was initiated. Over the course of 5 days the patient’s condition improved rapidly. Another EGD was performed and a follow-up biopsy showed significantly improved histological findings. She could be discharged after 2 weeks of hospitalization without any complications and with continuation of the eculizumab therapy. However, since the kidney transplant was a probable cause of the disease, the patient was readmitted for a planned nephrectomy 2months later after complete recovery. No signs of recurrence of her thrombotic microangiopathy had occurred in the meantime.\nAfter successful nephrectomy, histopathological findings were dominated by severe chronic tissue scarring with more than 90% interstitial fibrosis and tubular atrophy and severe inflammation with lymphoplasmacellular and granulocytic purulent interstitial nephritis and pyelitis as well as acute cellular rejection (Banff II). In addition, preglomerular arterial alterations were found going in line with previous and still mildly active thrombotic microangiopathy (Fig. g,h). Therefore, kidney pain and subfebrile temperatures prior to the first admission were likely due to severe renal inflammation. |
A 51-year-old male with a history of nonischemic cardiomyopathy with a left ventricular assist device was admitted for expedited heart transplant evaluation. The evaluation included an elective colonoscopy in light of a family history of colorectal cancer in his mother who died at age 61 from the disease. The patient had his first screening colonoscopy at age 45 and was diagnosed with benign polyps, which were removed, and left-sided diverticulosis. The procedure was uncomplicated and he was advised to repeat a colonoscopy in five years.\nThe patient was without GI symptoms at the time of his colonoscopy. He denied tobacco, alcohol, or illicit drug use. His medications included amiodarone, aspirin, famotidine, levothyroxine, lisinopril, metoprolol, sildenafil, and intravenous heparin as well as torsemide, acetaminophen, docusate sodium, and melatonin as needed. On examination, he had a left ventricular assist device port entering at the upper abdomen, but otherwise the abdomen was soft and nontender to palpation with normal bowel sounds and no appreciable masses or ascites.\nThe patient underwent a standard bowel preparation which included a clear liquid diet the day prior to the procedure and 20mg of Dulcolax with 4 liters of polyethylene glycol the night prior to the procedure. Monitored anesthesia care sedation was administered with propofol. The colonoscopy was performed at night without difficulty with good bowel preparation. Abdominal pressure was briefly required to maneuver around the splenic flexure. The colonoscope was advanced to the cecum with identification of the appendiceal orifice and ileocecal valve. Findings included multiple sigmoid and descending colon diverticula and two, small (<5mm) sessile polyps that were removed using cold forceps.\nThe night of the procedure the patient had no pain or nausea and ate dinner and breakfast the following morning without incident. He then developed epigastric abdominal pain in the midmorning approximately 12-14 hours after the procedure and had one episode of nonbloody, nonbilious emesis following lunch. On physical examination, he was afebrile with a blood pressure of 104/89mmHg, heart rate of 68 beats per minute, and oxygen saturation of 100% on room air. Abdominal examination was notable for mild distension and moderate tenderness to palpation in the epigastric region without guarding or rebound tenderness and decreased bowel sounds. Laboratory examination revealed an elevated lipase of 2275 U/L and amylase of 1141 U/L. Additional abnormal laboratory findings included an elevated aspartate aminotransferase of 105 U/L, alanine aminotransferase of 94 U/L, and total bilirubin of 1.4 μmol/L (normal prior to the procedure). An abdominal X-ray did not reveal an obstructive bowel gas pattern or evidence of free air. A computed tomography (CT) scan of the abdomen/pelvis revealed diffuse edematous changes of the pancreas with surrounding inflammatory stranding in the bilateral paracolic gutters, extending superiorly to the perihepatic region and inferiorly to the pelvis (). The constellation of symptoms, labs, and imaging were suggestive of an episode of acute pancreatitis.\nThe patient was treated conservatively with bowel rest, intravenous fluids, and analgesics as needed. Over the next three days his symptoms and abdominal examination improved and his diet was advanced to a regular diet. The lipase normalized to 15 U/L. |
These anatomic findings were modified slightly and employed clinically. The patient is a 53-year-old man who presented to a level 1 trauma center after being involved in a motor vehicle crash. He was the unrestrained driver of a car that crashed into a tree. He had multiple left-sided rib fractures with a flail segment and hemothorax. The patient’s respiratory status did not improve during the course of 3 days with maximal multimodal pain management including an epidural pain catheter and aggressive pulmonary toilet. He had persistent spells of sharp incapacitating pain along his left chest and could not pull volumes greater than 300 mL on incentive spirometry.\nA 3D reconstruction was done on his admission CT scan (). His left ribs were fractured at multiple levels: ribs 2 and 3 fractured posteriorly but not displaced, rib 4 fractured posteriorly with displacement, rib 5 fractured in a flail pattern laterally and posteriorly, and rib 6 fractured laterally with displacement. After informed consent was obtained, he was taken to the operating room for evacuation of hemothorax and chest wall stabilization.\nThe patient was placed in the right lateral decubitus position. A small incision was made posteriorly along the inferior aspect of the scapula. The dissection was carried down to the chest wall and a plane was developed with blunt dissection using a sponge stick. A Bookwalter was used to exposure placing a long Richardson blade underneath the scapula to develop the operative filed. A separate incision was made and a trocar was placed in the midaxillary line through which a laparoscope was introduced. The posterior fractures on ribs 4 and 5 were visualized. They were reduced and the anterior surface of the ribs close to the fractures was cleaned off. The fractures were bridged and plated with 50 mm Acute Innovations RibLoc plates. Identification of the anterior-lateral fractures proved difficult as the fracture on fifth and sixth ribs was directly under the anterior serratus muscle. The laparoscope was removed. A separate incision was made directly over the anterior-lateral fractures on ribs 5 and 6. Two 50 mm plates using standard open technique. Prior to closure, the left hemithorax was drained thoracoscopically and a chest tube was left in place.\nThe patient was brought to the ICU postoperatively. A chest radiograph was done to confirm adequate drainage of the pleural space. He was maintained on a ventilator for 48 hours and was extubated to high flow nasal cannula. During the course of the next 24 hours he was weaned to room air. His chest drain was removed. His pain was negligible and he was walking the halls independently. He was discharged on postoperative day 4. On his follow-up appointment, he continued to feel well without any disabling symptoms.\nThe patient went to the operating room on postopertive day 5. By this time the tissues appeared more hemorrhagic which made visual identification of the fractures challenging. Also, there was more oozing from the tissues as the traumatic inflammation of the tissues had increased the degree of vascularity. This resulted in more tissue bleeding adding to the difficulty visualization of the injury.\nThere is yet no standardization of chest wall injuries. The American Association for the Surgery of Trauma Abbreviated Injury Scale chest wall injury score is inadequate to describe the injury for operative management. We propose the following ():\nAnterior: from the sternum to the anterior axillary line.\nLateral: from the anterior axillary line to the posterior axillary line.\nPosterior-lateral: from the posterior axillary line to the angle of the rib.\nPosterior: from the angle of the rib to the head of the rib.\nThe importance of defining the injury patterns is critical as it dictates whether or not a particular surgical approach will be effective. We determined that the VARP approach is ideal for posterior-lateral injuries specifically for the subscapular rib fractures. Lateral rib fractures can also be performed. Our patient had rib fractures on the boarder of the anterior and lateral aspect. This required a separate incision to address these injuries.\nLike any new procedure, many of our current surgical instruments are not specifically designed for this type of surgery. |
A 54-year old Thai woman presented to our facility with a two-month history of urinary incontinence induced by coughing.\nShe had a history of cervical squamous cell carcinoma, stage IIb, which was treated 15 years previously with radiation therapy. Her six-month interval follow-up pelvic examination and cervicovaginal PAP smears had revealed negative findings and the disease had been considered as in complete remission by the attending radiologist. She was first referred to our gynecology department to find out the cause of her incontinence. On pelvic examination by a gynecologist, her uterine cervix was found to be atrophic with an extraluminal compression at the anterior vaginal wall, covered by an intact vaginal mucosa. Laboratory investigation results revealed mild hematuria (3 to 5 red blood cells per high-power field) and mild anemia (hemoglobin 10 g/dL, hematocrit 31.1%). Eventually, our patient was referred to our urology department for further management.\nDuring the process of the Q-tip test, the applicator was passed with difficulty and deviation to the right. Cystoscopy was performed. Her urethra showed no meatal stenosis and unremarkable mucosa, but the device was passed with difficultly due to external compression from the left side of the proximal urethra. Her bladder had a large sessile mass on the left lateral wall with extension to the bladder neck. Both ureteral orifices could be identified clearly with unremarkable appearance and efflux. Clinically, the differential diagnosis included recurrent cervical cancer, bladder cancer and retroperitonal sarcoma. Urine cytology and transurethral resection of the bladder tumor were performed, with a negative cytology report and a pathological diagnosis of high-grade urothelial carcinoma. A computed tomography scan demonstrated a heterogenous enhancing mass, 5.3 × 6.5 × 6.0 cm, at the left inferior-lateral wall of the bladder with invasion of the uterus and attachment to the left pelvic side wall, as shown in Figures and . There was no hydronephrosis, hydroureter, liver mass or lymphadenopathy seen radiologically. Subsequently, anterior pelvic exenteration and ileal conduit with pelvic lymphadenectomy, as well as an incidental appendectomy, were performed.\nGross pathological examination revealed a large mass on the left lateral wall that measured 7.0 cm across its greatest dimension. The surface of the tumor on the mucosal side was smooth. The mass obviously protruded from the serosal surface of the bladder and was attached to the uterus, but could still be separated from the organ with ease.\nMicroscopically, the overlying urothelium was intact with no dysplastic change (Figure ). The underlying mass was composed of highly pleomorphic spindle-to-polygonal shaped neoplastic cells with frequent mitotic figures and many atypical ones (Figures and ). Their cytoplasms ranged from clear to pale on eosinophilic staining. Many cells contained bizarre-shaped nuclei with focal multinucleated giant cells. No definite component of urothelial carcinoma was seen. An immunohistochemical study was performed, showing that the neoplasm was immunoreactive for vimentin (Figure ) and CD68 (Figure ), but negative for epithelial marker (AE1/AE3) and other markers for soft tissue tumors (desmin, smooth muscle actin and S-100 protein). A CD10 test was also performed for exclusion of carcinoma of renal cell origin, and yielded a negative result. Therefore, a diagnosis of malignant fibrous histiocytoma was made.\nPathological findings from other organs, including the uterine cervix, were unremarkable. No residual cervical dysplasia or neoplasm was detected. All 12 lymph nodes showed no neoplasm. The disease was clinically and pathologically staged as T4b N0 M0.\nAfter surgery, our radiation therapist advised not providing additional radiation therapy in the pelvic area. Our patient, therefore, received a course of adjuvant chemotherapy (doxorubicin and ifosfamide). However, two months after the surgery, a bony metastasis was detected at the L5 spine level, and palliative radiation therapy in that area was suggested. Despite aggressive surgical treatment along with adjuvant chemotherapy and palliative radiation therapy, our patient developed further locally recurrent disease with invasion to the right pubic bone and rectum. At this time, a multidisciplinary approach for palliation of our patient's symptoms is still in process. |
A 40-year-old female, with 3-month history of nasal obstruction and tinnitus was admitted in August 2012. Nasopharyngeal endoscopy and biopsy already had been performed in another hospital, showing nonkeratinizing undifferentiated NPC. This histopathologic diagnosis was confirmed in our center. Magnetic resonance imaging (MRI) of the nasopharynx and neck revealed the tumor was confined to the nasopharynx and the bilateral locoregional cervical lymph nodes enlarged with its greatest dimension of 2 cm. Chest computed tomography (CT) scan, ultrasound of abdomen, and whole-body bone scan ruled out distant metastases. So clinical staging was determined to be T1N2M0, IIIA according to American Joint Committee on Cancer TNM Staging System for NPC (7th ed, 2010).\nThe patient was treated with definitive IMRT to 7050 cGy for primary tumor and 6600 cGy for infiltrated regional lymph nodes. Concurrent chemotherapy based on cisplatin and 5-flurorouracil was administrated for 2 cycles and then 2-cycle chemotherapy was given subsequently to consolidate the efficiency with the same regimen. At the end of therapy, she obtained clinical complete response by nasopharynx and neck MRI.\nIn the initial therapy, the patient had undergone abdominal ultrasonography for 4 times, and no hepatic lesions were noted during this period. Nevertheless, when she came to our hospital for 3-month conventional follow-up in April 2013, abdominal sonogram revealed a liver cystic lesion with thin wall and smooth margin of 18 × 16 mm in the right liver lobe, and the lesion was interpreted as a simple liver cyst (Fig. ). Therefore, intense follow-up was suggested. Five months later, the cystic lesion enlarged to be 59 × 46 mm, with thick wall, but no signal of blood flow. Further CT presented a low density and heterogeneous lesion taking irregular wall and incomplete septa with strong contrast enhancement, indicating liver abscess (Fig. ). However, the patient was asymptomatic, with no fever, no right up abdominal pain, and no palpable mass by physical examination. On the contrary, laboratory findings were negative, liver function was normal, the white blood cell count, C-reactive protein, and cancer-related antigen including α-fetoprotein were within the normal limits. Afterward the patient was transferred to another hospital for abscess drainage, and fluid culture was negative, but fine-needle aspiration was not performed. After drainage, the mass shrank significantly to one-third of the original size. However, it returned to previous size by CT 2 months later. Thus, the liver lesion was suspected to be malignant. Work-up examination including chest CT, pelvic MRI, emission CT for bones, gastroscopy, colonoscopy, and brain MRI excluded other lesion that may account for another primary tumor or extra-hepatic distance metastases from NPC. In addition, nasopharynx MRI showed no evidence of local relapse. On November 21, 2013, surgical resection was administrated both for histologic diagnosis and treatment, and the surgical margin was negative. Histopathologic examination definitely confirmed that the metastasis originated from NPC, since the cells of the surgical segment were similar to primary NPC on the morphology and they were positive in Epstein–Barr virus (EBV) encoded RNAs (EBERs) (Fig. ). No adjuvant chemotherapy was done after resection of the liver metastasis.\nHowever, relapse-free survival time lasted only for 8 months. In July 2014, 2 small cystic lesions were found on abdominal CT scan again, which were extremely similar to simple cysts (Fig. ). Further contrast-enhanced ultrasonography provided no sign of “fast in and fast out,” that is, a characteristic appearance in malignant carcinoma. As a result of multidiscipline team discussion, “watch and wait” strategy was recommended. However, the number of hepatic lesions increased to 4, and the size of previous 2 cysts enlarged to be 3 cm within 3 months. The multidiscipline team members reached a consensus that the cystic lesions were metastases from NPC and suggested a palliative systematic chemotherapy. Then the patient received chemotherapy with paclitaxel and cisplatin for 6 cycles. During the first 3 cycles, partial lesions diminished in size, but stayed stable within the later 3 cycles. Nevertheless, the intraliver metastases exhibited significant progress soon after the chemotherapy. Subsequently, she underwent chemotherapy with gemcitabine plus targeted therapy with nimotuzumab, then single navelbine both for 3 cycles, but neither protocols showed notable effects on hepatic lesions. In addition, during the phase of chemotherapy with navelbine, the patient complained of chest pain, an irregular and fixed lump was found on her chest wall, approximately 3 cm in diameter. After local surgical resection was performed, the lump histologically was demonstrated to be an extra-hepatic metastasis from NPC. Considering the patient's good performance status score and grade A liver function according to the Child-pugh grading system, transcatheter hepatic artery chemoembolization (TACE) was administrated for 5 times from December 2015 to April 2016. After the TACE treatment, all the lesions were obviously decreased in size, with the largest metastasis decreasing from 64 × 53 to 33 × 32 cm (Fig. ). Meanwhile, carbohydrate antigen 125 (CA125) decreased from 125 to 60.8 U/mL, and squamous cell carcinoma antigen decreased from 2.8 to 0.9 ng/mL. Then 4 cycles of gemcitabine plus cisplatin were offered to consolidate the clinical effects. In the next 6 months, her liver metastases maintained stable, but lung metastases were noted. In October 2016, the CT showed the liver metastases progressed. At last, she died of liver failure in March 2017. |
A 64-year-old Caucasian woman with a medical history significant for morbid obesity, intravenous and subcutaneous drug abuse, chronic pain, hepatitis C, diabetes mellitus type 2 on insulin, multiple episodes of abdominal cellulitis with methicillin-resistant Staphylococcus aureus, dyslipidaemia and essential hypertension presented to the emergency department complaining of intermittent lower left-sided chest and shoulder pain for the past 6 weeks. She described the pain as sharp in nature, 10 over 10 in severity, localised to the left lower side of the chest with radiation to the left shoulder tip. She said that her pain got worse with a deep breath or any movements. She denied any previous history of similar pain. She reported feeling nauseated with multiple episodes of non-bilious, non-projectile, non-bloody vomiting. Her review of systems was positive for decreased appetite, subjective fever with frequent rigours and sweats. She denied measuring her temperature at home. Social history was significant for intravenous cocaine and subcutaneous morphine abuse. Family history was positive for premature coronary artery disease in her brother and pancreatic cancer in her son. On physical examination, her vital signs were as follows: blood pressure 175/103 mm Hg, heart rate 113 beats per minute, temperature 36.7°C, respiratory rate 22 per minute and oxygen saturation 98% on room air. Her oral mucosa was dry. There was reproducible chest tenderness in the left lower side. Heart sounds S1 and S2 were heard without any murmurs, rubs or gallops. Lungs were clear to auscultation with good air entry bilaterally. Her abdomen appeared normal on gross inspection. Normal bowel sounds were heard in all four quadrants. There was no reproducible abdominal tenderness on superficial or deep palpation. In view of her risk factors, she was admitted to rule out acute coronary syndrome. She was kept overnight for observation on a telemetry floor where she continued to have fever spikes as high as 38.9°C the following day. She also reported tenderness in the left upper abdomen the next day. |
A 75-kg, 61-year-old man, a well-controlled hypertensive was scheduled for laparoscopic nephrectomy for right renal cell carcinoma. General anesthesia was induced with intravenous (IV) fentanyl 150 mcg and propofol 200 mg, and thereafter maintained with isoflurane 2% in oxygen and air. His trachea was intubated after establishing muscle relaxation with IV vecuronium 8 mg and repeat doses of 1 mg were administered to maintain a train-of-four count of 0 to 1. Patient was mechanically ventilated with an initial setting of 600 ml of tidal volume and a respiratory rate of 12 per minute. He was positioned for surgery in the left modified kidney (flank) position. Intra-abdominal pressure (IAP) was maintained less than 15 mmHg during laparoscopy. Maximum peak airway pressure noticed during laparoscopy was 25 cm of H2O. End-tidal carbon dioxide (ETCO2) less than 40 mmHg was maintained by increasing the respiratory rate to 15 to 16 per minute. Urine output was 100 mL in the first hour followed by a total of 20 mL in the next 4.75 hours despite two fluid challenges with 250 mL of 6% hydroxyl-ethyl starch (HES). Throughout the procedure the systolic and diastolic noninvasive blood pressures were 90 to 100 mmHg and 50 to 60 mmHg, respectively. There was a transient drop to 78/40 mmHg immediately after positioning, which was treated with a bolus of IV mephentermine 6 mg. His heart rate ranged from 60 to 90 beats per minute. There was no episode of desaturation during the procedure. A total of 1.5 L of Ringer's lactate and 1.0 L of 6% HES were infused during the entire 5.75-hour procedure. Intraoperative blood loss was approximately 50 mL. After the procedure with the patient supine, neuromuscular blockade was reversed after noting four twitches on train-of-four count. The trachea was extubated when he was awake, breathing adequately with good cough reflex and having a sustained head lift. He was monitored in the postoperative room and administered oxygen supplementation 6 L/min by face mask.\nApproximately 1 hour after tracheal extubation, he became anxious, complained of discomfort over the left chest and was tachypneic. The SpO2 was 77% and coarse crepitations and rhonchi were heard over the left chest. Treatment was initiated by enhancing oxygen supplementation (by changing to a venturi mask set to deliver 0.6 FiO2) and IV furosemide. Blood gas analysis at 0.6 FiO2 revealed poor oxygenation with metabolic acidosis [pH: 7.288, pCO2: 39.9 mmHg, pO2: 47.7 mmHg, SaO2: 80.1%, HCO3: 18.2(std), 18.5(calc), ABE: -7.3(calc), 6.9(std)]. ECG revealed sinus tachycardia with S1Q3T3 pattern suggestive of right ventricular strain. A bedside echocardiogram showed normal biventricular systolic function. Troponin T level was not elevated at 12 hours after the episode. The chest X-ray revealed haziness of the entire left lung fields, more so in the mid and lower zones and haziness in the right perihilar area [] suggestive of dependent lung edema. He was shifted to the ICU and noninvasive ventilation (NIV) initiated in the propped up position. Clinical and radiological improvement [] was noted after 12 hours and the NIV was discontinued. The rest of his hospital stay was unremarkable. |
Mrs. E was a 57-year-old lady with a 12-year history of COPD, with an FEV1 of 30 %. Furthermore, she had a past medical history of carcinoma of the breast, type 2 diabetes mellitus and left ventricular failure (LVF), which was diagnosed on an echocardiogram (ECHO) in July 2014. Despite a diagnosis of COPD she continued to smoke 20–30 cigarettes/day.\nShe was prescribed a number of medications to help manage her airways disease including tiotropium, seretide 500, salbutamol, prednisolone and most recently oral theophyline (450 mg twice daily), which was started in April 2013 as an adjunct to her current medications.\nShe was admitted in June 2015 following a fall with notable decreased sensation bilaterally in her lower limbs and a tachycardia of 133 beats per minute. Spinal cord compression was an initial concern, however this was excluded with a magnetic resonance imaging (MRI) spine. There was no change to her theophylline prescription on admission to hospital and no new medication was prescribed that would affect theophylline metabolism.\nOver several days additional symptoms were noted, including worsening nausea, blurred vision, increasing fatigue (with stable blood sugars) and worsening speech with slurring of words. Development of vomiting resulted in a decision to measure her theophylline concentration and perform a CT head, as no obvious cause for her symptoms had been elicited. The CT head was reported as clear of any ischaemia, haemorrhage or space-occupying lesion.\nThe trough theophylline concentration was 59.3 mg/l. The last result obtained in July 2014 during an admission for influenza related infective exacerbation of COPD was reported as 20.2 mg/l, marginally above the 10-20 mg/l recommended reference range. The concentration was then not re-checked either in primary care or in hospital; it therefore became apparent this was a case of chronic theophylline toxicity.\nThe medical team at The National Poisons Information Service was contacted for advice on management of this patient. The rationale being abnormally high theophylline concentration, furthermore this was a case of chronic, rather than acute toxicity, for which we felt specialist input was required.\nThe patient was monitored for significant effects of toxicity; these include seizures (reported at trough concentrations of 50 mg/l []), reduced conscious level and tachyarrhythmias. Although Mrs. E suffered a persistent tachycardia, her heart rate remained in sinus rhythm. Cardiac monitoring was started. She was managed conservatively by stopping the theophylline and monitoring symptoms. Due to vomiting, oral activated charcoal was excluded and although charcoal haemoperfusion was discussed, it was not accessible at the RUH and as Mrs. E was haemodynamically stable and not acidotic, it was deemed not to be essential. This point will be discussed further in the conclusion.\nMrs. E developed electrolyte imbalances not long after the vomiting started, suffering hypokalaemia (3.2 mmol/l), hypomagnaesemia (0.56 mmol/l) and hypocalcaemia (1.96 mmol/l). This was postulated to be secondary to both vomiting and theophylline accumulation. This subsequently resulted in a pseudo-obstruction, diagnosed on abdominal x-ray. A nasogastric tube was placed and she was supplemented with appropriate electrolytes via intravenous infusion. An arterial blood gas demonstrated a pH 7.398 (7.34-7.44), pO2 (kPa) 9.24 (11–14), pCO2 (kPa) 6.36 (4.7-6.0), HCO3− (mEq/l) 21.7 (22–26), lactate (mmol/l) 1.5 (0.4-2.2). As no signs of acidosis were present and bicarbonate was at the low end of the normal range, we decided not to prescribe bicarbonate for this patient.\nAfter stopping her theophylline and without any active intervention her serum concentration fell gradually (Table ). By the time her theophylline concentration was within therapeutic range, her electrolytes had been corrected and her pseudo-obstruction had resolved. However, she developed signs of fluid overload, her heart rate also remained in persistent sinus tachycardia. Notably, findings of Congestive Cardiac Failure (CCF) were demonstrated on a previous echocardiogram and were substantiated by results of her computed tomography (CT) abdomen/pelvis and ultrasound sonography scan (USS) liver during her current admission; these both showed abnormal appearances of the liver consistent with cardiac dysfunction. It therefore appeared she had developed acute CCF. Ultimately, this resulted in challenging fluid balance management with serial chest x-rays demonstrating worsening bilateral effusions and renal function continuing to decline until the death of the patient. |
A 51-year-old woman was transferred from a nearby hospital after receiving heavy pretreatment for disseminated breast cancer. At the time of admission to our hospital in July 2017, the patient’s general condition was poor, with an Eastern Cooperative Oncology Group (ECOG) performance status of 4. She complained of widespread skin metastases with discharge and generalized edema (). According to her medical history, the patient was diagnosed with inoperable advanced TNBC and underwent 10 cycles of palliative chemotherapy with docetaxel and doxorubicin from May to December 2015, resulting in a partial response. However, the treatment was terminated owing to intolerance of the cumulative doxorubicin dose. Thereafter, the patient underwent a palliative modified radical mastectomy in December 2015. With ongoing disease progression, she received palliative radiotherapy and multiple lines of chemotherapy, including paclitaxel, gemcitabine, capecitabine, vinorelbine, and eribulin, before visiting our hospital.\nAt the time of admission, the patient’s vital signs were stable and she was alert. Her chest computed tomography (CT) scan demonstrated a diffuse infiltrative mass lesion in the anterior chest wall with bilateral pleural effusion and multiple metastatic lymphadenopathies in both the neck and axilla. The abdomen-pelvis CT scan revealed multiple liver, lymph nodes, and abdominal wall metastases. Tissue biopsy of the right chest wall tumor mass identified the tumor as a triple-negative-type invasive ductal carcinoma (). Cisplatin monotherapy was selected based on the results of several pilot studies, which revealed a remarkable efficacy of cisplatin for TNBC [,]. We also took into consideration the patient’s poor general condition and the mild cisplatin-induced toxicity. The patient received 75 mg/m2 of cisplatin every 3 weeks, along with standard hydration and antiemetic prophylaxis. Chemotherapy was administered in an in-patient setting because of the patient’s poor general condition and the extensive dressing care needed for the metastatic skin lesions. After three cycles of cisplatin, the occurrence of skin ulcerative lesions was remarkably decreased. As the patient’s ECOG performance status was dramatically improved to 1, the treatment was continued in an outpatient setting. After six cycles of the regimen, responses were further noted in both the liver and skin lesions (). With regard to adverse events, transient neutropenia and mild nausea were identified, yet both were manageable. The patient recently completed her 17th cycle of cisplatin with a good performance and minimal peripheral neuropathy ().\nAlthough she has no family history of breast or ovarian cancer, germline and somatic BRCA mutation tests were performed in the peripheral blood and primary/residual malignant tumor tissues for the potential use of a PARP inhibitor. However, no pathogenic BRCA mutation was identified ().\nThe patient provided written informed consent for publication of clinical details and images. |
A 17-year-old girl, weighing 50 kg with a history of delayed developmental milestones, had presented with a right frontal swelling [] associated with headache of 3-year duration. She underwent only partial excision of the same swelling 2 years ago in view of excessive bleeding during surgery. Moreover, complete excision of a similar swelling diagnosed as fibrous dysplasia over the contralateral parietal eminence was done 5 years back. On examination, her neurocutaneous markers suggested classical KTS with presence of distinct, linear bordered port-wine stains [] over her trunk, lower back and extensor aspects of bilateral upper and lower limbs and multiple small subcutaneous firm swellings (neurofibromas) over the right thenar eminence, right olecranon and dorsal spine at D4-D10 level. She had lymphoedema of both the lower limbs and disproportionately enlarged feet with clubfoot deformity []. All these features were reported to be present since birth, with exacerbation of these manifestations following the attainment of menarche at 12 years of age. Magnetic resonance imaging (MRI) demonstrated right frontal calvarial haemangioma measuring 8 cm × 9 cm × 5 cm with intracranial extension compressing the ipsilateral frontal lobe [] and associated Chiari 1 malformation []. The cerebral angiogram revealed increased vascularity of the lesion with multiple feeders predominantly from the right middle meningeal artery. Preoperative embolisation was considered but not performed in view of concerned risk of cortical infarcts in the presence of multiple feeders. Ultrasound examination of the abdomen excluded hepatic haemangiomas and other solid organ involvement. In addition, cardiac workup ruled out associated congenital heart disease. Her chest X-ray revealed left hemidiaphragm elevation with a possible eventration of the diaphragm. Blood investigations including coagulation profile were normal. She was scheduled for craniotomy and excision of the haemangioma. Preoperative preparation included continuation of levetiracetam 500 mg thrice daily, arrangement of adequate blood products in view of anticipated blood loss and written informed consent. As mask ventilation was easy, standard anaesthetic induction was performed. In view of Chiari malformation and anticipated soft tissue changes in the airway, intubation was performed using C-Mac videolaryngoscope at the first attempt. Soft tissue overgrowth in the region of palate was observed during intubation. The patient was positioned carefully and craniotomy was performed []. Balanced anaesthesia was used with sevoflurane and intermittent boluses of opioids and muscle relaxants. Intraoperatively, 1.5 L of crystalloids (plasmalyte) were administered with adequate urine output obtained. The intraoperative blood loss was 2500 mL over 3-hour duration of surgery and this was replaced with 5 units of packed red cells, 3 units of fresh frozen plasma and 2 units of platelets. Extubation and recovery from anaesthesia were uneventful. |
A 52-year-old male patient complained of pleuritic pain of the right chest, a persistent cough, and yellowish blood-tinged sputum for two months. He had been diagnosed with pulmonary tuberculosis and completely recovered after medical treatment; he had no other underlying diseases. The patient was a heavy daily drinker and had lost 7 kg over the previous six months before admission to the hospital. His blood pressure was 125/80 mmHg, pulse rate was 100/min, and body temperature was normal. Right breathing sounds were decreased with crackles. Chest radiographs showed old tuberculosis scars and a newly developed empyema in the right lung field (). Tomography showed several cavitary lesions of different sizes, some of which had fluid collection, which suggested an infected cyst, an old tuberculosis scar, or active tuberculosis. Partial calcification of the pleura, pleural thickening, and pleural effusion were also found (). A yellowish substance was located in the lumen of the right upper lobe apical segmental bronchus at bronchoscopy and a pathologic examination of the substance was performed. The pathologist reported the substance to be pulmonary aspergillosis ().\nThe empyema was drained through a percutaneous catheter and air leakage was observed. Amphotericin B (AMB) was injected after the patient was diagnosed with pulmonary aspergillosis. After the administration of AMB, a fever that the patient had since the second day of admission subsided, the white blood cell count decreased from 12,900/mL to 7,900/mL, and C-reactive protein decreased from 22.9 mg/dL to 4.4 mg/dL. On the fourteenth day of admission, a 3 French Fogarty catheter was introduced to the right upper lobe apical segmental bronchus and the bronchus was obliterated with the balloon under general anesthesia. After the obliteration of the bronchus, the air leakage decreased dramatically. The location of the fistula was confirmed with a fistulography by using a percutaneous catheter and a blockage test with the balloon of the Fogarty catheter. We attempted fistula occlusion with BioGlue and a coil while the bronchus was blocked with the Fogarty catheter balloon, but the occlusion of the fistula failed due to the existence of multiple broncho-pleural fistulae.\nNutritional support and AMB injections continued for 1 month and during that period the patient gained 3 kg and his nutritional state improved. Pleural cavity irrigation with AMB mixed with 1 L of 5% dextrose was performed for 5 hours daily (the total amount of AMB was 1,300 mg for irrigation). However, the patient developed an intermittent fever again. Since the persistent air leakage and drainage of turbid fluid through the percutaneous catheter lasted for 1 month, a lobectomy and pleural decortication was performed. No air leakage was observed and the fever subsided after the operation. A pathologic exam showed Aspergillus invading the pleura.\nTwo weeks after the operation, the patient developed a fever, leukocytosis, and air leakage. Computed tomography showed an air space at the posterior pleural cavity. Irrigation with 12,000 mL of normal saline was performed in the operating room and the absence of air leakage through the bronchus stump was confirmed. However, air leakage was observed from the surface of the lung. The antifungal agent was converted to an oral voriconazole, and the patient was discharged with one chest tube to which a Heimlich valve was connected since there was air leakage and a small amount of a yellowish substance drained through the chest tube.\nThe patient developed massive bleeding (1,000 mL) through the chest tube 5 months after being discharged. The intra-operative finding was that the bleeding originated from the chest tube tract. Bleeding control and myoplasty with the pectoralis major muscle and latissimus dorsi were performed to fill the dead space of the pleural cavity. There had been no dead space observed immediately after the operation, but dead space in the pleural cavity had eventually formed. However, the dead space gave rise to no clinical problems. The patient was discharged without the chest tube and no complications have arisen for 15 months post-retreatment. |
Two years before, a 59-year-old Japanese woman was referred to our department because of a mental anxiety and intense pain in the left tongue after the extraction of the right mandibular third molar 17 years ago. To manage such a serious perception abnormality, she had received stellate ganglion block (SGB) ten times and medical treatment in various psychosomatic medicine and psychiatry and received various medication of psychotropic drugs for more than 3 years. Without any effectiveness, she suddenly had dropped in panic disorder and depression many times.\nOn her first visit to our Department of Oral and Maxillofacial Surgery, Wakayama Medical University, we checked the details for the tongue perception with various medical examination methods for the first time.\nOur neurosensory assessments included the following.\nThe subjective assessment done was the rating of the subjective sensation (visual analog scale) of the affected area, which was the most seriously bad limited right point.\nBrush stroke directional sensation with camel hair brush (brush); horizontal, vertical, and rotational stimulating movement were applied (0 means recognized not at all, 1 means recognized only one direction, 2 means recognized two directions, and 3 means recognized all movements): the left anterior region of her torn tongue revealed 3 but the posterior region indicated 0. Pinprick test: sharp touch reaction with needle, which was indicated in poor reaction. Static two-point discrimination (2PD): the left anterior region reacted in 8 mm but the left posterior region reacted in 15 mm. Pressure pain threshold: the Semmens-Weinstein monofilament (SWM), composed of 20 different diameter monofilaments: 1 was assigned to the smallest diameter and 20 was the largest diameter monofilament. With each instrument, the anterior region of her torn tongue reacted with 5 and the posterior region reacted with 8. Thermal discrimination (thermal): hot and cold sensation; hot water 42 °C; cold sensation, ice 0 °C, pin prick. Both her thermal reactions were positive. Tinel’s reaction on the torn lingual nerve: against this reaction, she received a violent sharp stabbing pain along the left side of her disturbed tongue. Gustatory sensation assessed with localized testing discs (Sanwa Kagaku Kenkyusho, Japan): salty, sodium chloride 1 mol/l; sweet, sucrose 1 mol/l; sour, acetic acid 0.4 mol/l; bitter, quinine 0.1 mol/l, which were absent for all reagents on the injured side of her tongue. All the abovementioned inspections were performed at pre-operation and at 6 months after the microsurgery retrospectively. The outcome was analyzed with Sunderland grade and by the Medical Research Council Scale (MRCS). Summarizing such inspections of careful tests, we diagnosed this case as an old serious perception abnormality in the left LN. The detailed data is presented in Table .\nAfter taking informed consent with patient and family, we decided to enforce the lingual nerve reconstruction with allograft nerve microsurgically.\nMicrosurgical treatment of LN injury was performed under general anesthesia. The LN was exposed through an intraoral mucosal incision. A large granuloma of LN adjacent to extracted cavity of third molar teeth was revealed. The granuloma and peripheral neuroma surrounding the torn LN about 14 mm length were removed completely (Fig. ). Between each end of lingual nerves, allograft nerve (RENERVE®) about 18 mm length was inserted and sutured with 8-0 nylon in the microsurgical field (Figs. and ).\nAt 6 months and 1 year after the operation, the patient showed an amazingly improved sensory recovery reaction. The data of SWM test, 2PD, Tinel’s reaction, and gustatory sensations improved remarkably (Table ). It is incredible 1 year later of the microneurosurgical operation; she was free from psychotropic drugs except for a sleep inducer and regained a sound healthy daily life. |
A 73-year-old Caucasian woman was scheduled to undergo elective colonoscopy. She had no history of gastric reflux or any other record of an upper gastrointestinal chronic or acute disease. Her known medical history consisted of mild hypertension, a prosthetic hip joint, and colon diverticulosis.\nIn preparation for the colonoscopy, at home she received soluble MoviPrep powder bags. Anti-emetic medication was not prescribed. MoviPrep is an osmotic laxative whose main component is polyethylene glycol (PEG-3350) and which also contains sodium sulfate, sodium chloride, potassium chloride, sodium ascorbate, ascorbic acid and the additives aspartame, acesulfame-potassium, orange/lemon aroma, maltodextrin and sugar. She followed the manufacturer's instructions and performed the first colon lavage in the afternoon of the day prior to the day of examination by drinking the first 1000 ml of MoviPrep solution in portions of 200 ml ea. as well as drinking the corresponding amount of 1000 mL of water within two hours. The first colon lavage was successful and uneventful. In the early morning on the day of colonoscopy she started the second colon lavage by drinking two portions of 200 mL of PEG solution. and the appropriate additional amount of water. A few minutes later she suddenly became nauseous and was forced to vomit excessively. At the same time she felt a sudden pain in the middle of her back below the left scapula region. Her relatives called the emergency services and she was transferred to the next county hospital. In the emergency room she presented with signs of an acute abdomen (abdomen tender and bloated) and persistent and slightly increasing back pain. An immediate computed tomography (CT) scan with oral contrast medium (CM) was conducted and showed a CM extravasation at the level of the lower thoracic esophagus just above the esophagogastric junction and a small mediastinal emphysema. Boerhaave syndrome was diagnosed and the woman was transferred to the nearby university hospital for further treatment. After transfer, an endoscopic evaluation was conducted and only a small longitudinal laceration (length approximately 15 mm) just above the Z line on the left side of the esophagus was seen and suspected as the site of perforation (Figure , arrow). The lesion was not visible when evaluated in inversion from the gastric side (Figure ). Therefore, we initially considered the perforation to be treatable by endo-sponge vacuum therapy. A polyurethane VAC sponge (V.A.C.® GranuFoam™ Dressing, Kinetic Concepts, Inc. P.O. Box 659508 San Antonio, TX 78265) was placed endoscopically in the esophageal lumen at the height of the lesion and was connected via a small gastric tube (Figure ) to a VAC therapy unit (ActiV.A.C.® Therapy Unit, Kinetic Concepts, Inc. P.O. Box 659508 San Antonio, TX 78265) with continuous suction at 125 mmHg. She also received a thoracic drain to the left hemithorax. The sponge was left in place for approximately eight hours until a CT scan with oral CM was performed the next morning.\nThe CT scan revealed persistent and significant CM leakage with gradual abscess formation and advancing mediastinal emphysema. At the same time, the CT scan showed only a thin filament-like CM fistula between the para-esophageal CM depot and the lumen of the esophagus (Figure ). Furthermore, clinically our patient slightly deteriorated by developing fever and increasing back pain. Her C-reactive protein and leukocyte levels were also increasing. We assumed that the small longitudinal laceration had a valve-like configuration and thus would not allow the VAC system to have a sufficient abscess draining effect. Thus, we decided to switch from the endoscopic therapeutic approach to open surgery.\nDuring surgery, a small perforation of 3 mm in diameter was found just 2 cm above the cardia on the left side of the esophagus (Figure ). The intra-operative endoscopy verified the perforation at the location where it had been suspected earlier (ov, Overholt clamp). The lesion was repaired with five stitches of PDS 3-0 suture and Nissen fundoplication. The result of the repair was examined by control endoscopy (Figure ). During the post-operative course the CT-guided application of an additional pigtail drain for persistent left thoracic abscess formation was necessary. Other than that, her post-operative course was uneventful and our patient recovered completely. She was discharged from hospital three weeks after the initial incident. |
A 28-year-old man presented at a traumatic surgery unit in an outlying hospital, where an external rotation of the left leg and a patellar dislocation were detected. Examination also revealed clinical signs of a neurofibromatosis-1 (Figure ). The patient had stumbled doing his work as a cook and had fallen on his flexed left knee. Conventional radiography of the knee demonstrated an osteochondral flake near the medial patellar margin, whereas the femoral fracture remained unnoticed (Figure ). After reposition of the patellar dislocation, the joint was stabilized with an orthosis, and the patient was sent home.\nTwo days later, because of increasing pain and swelling of the left knee, his family doctor arranged further radiographic examinations with MRI and CT scans, revealing the Hoffa fracture. The patient was then referred to a medical center for traumatic surgery. Surgical treatment consisted of diagnostic arthroscopy, open reduction of the fracture, and internal fixation with three 40-mm headless compression screws (Figure ). The screws were placed in posterior to anterior and caudal to cranial directions. The anterior horn of the lateral meniscus was fixed to the joint capsule using FiberWire 2-0, and the ruptured retinaculum was fixed to the medial patellar margin with two Mitek anchors. A 3 × 3 cm tumor close to the articular capsule turned out to be a lipoma and not, as initially suspected, a neurofibroma.\nAfter surgery, the knee was stabilized with a knee orthosis, and partial weight-bearing was prescribed for 10 weeks. Fracture healing was regular. In <4 months after the accident, the patient returned to work without any orthopaedic therapeutic appliances. In this case, the occurrence of a femoral fracture after a low-velocity trauma was probably influenced by reduced bone quality because of an underlying NF1, but NF1 did not influence fracture healing. Two years after the operation, the range of motion of both knees was unlimited, and no osteoarthrotic changes were noticed. |
A 15-year-old Igbo female from Enugu, Nigeria, presented in our clinic with her grandmother, with a history of asymmetry of the breasts noticed 4 years ago. The right breast was noticed to be gradually increasing in size, to its present adult size, with no corresponding change in the size of the left breast. There was no preceding history of trauma, and no nipple discharge. The obvious asymmetry had been a constant source of anxiety and embarrassment for the patient, and also for her caregiver. They had sought treatment at various health facilities but presented to our clinic eventually. The past medical was not remarkable, and she was not on any previous medications. She achieved menarche at 13 years of age. Both parents are dead; she lives with the grandmother, and they are of low socioeconomic status. At presentation, they had intense fears of the need for a plastic surgical correction of the left breast.\nOn examination, she was apprehensive and visibly self-conscious; her vital signs were stable with temperature of 36.8 °C, pulse rate of 80 beats per minute and blood pressure of 90/60 mmHg. She had marked breast asymmetry, with hypoplasia of the left breast of SMR 11 (Figs. and ), while the right breast had a staging of SMR V. In addition, we noted a large, hyperpigmented patch extending from the left axilla and covering the left anterior chest wall, and spreading posteriorly. This had been present since birth, and had not been given much attention since it was not associated with pain or other asymmetric skeletal abnormalities. Neurological examination did not reveal any abnormality. Abdominal ultrasound and skeletal radiographic imaging were unremarkable.\nBased on her symptoms, diagnosis of Becker’s nevus syndrome was made. The diagnosis of Becker’s nevus syndrome is mostly clinical, based on the presence of a Becker’s nevus with ipsilateral breast hypoplasia or hypoplastic defects of the muscle, skin, or skeleton. In our patient, there was a Becker’s nevus with ipsilateral breast hypoplasia. This syndrome, belonging to the class of epidermal nevus syndromes, is very rare, and is usually benign. She was placed on spironolactone tablets (50 mg daily). After 6 months of therapy, there was an improvement in the size of the hypoplastic breast; increased from SMR 11 to SMR 111 (Fig. ), and her fears were allayed. |
A 13-year-old boy presented to the emergency unit with inability to stand or walk. Eight days previously, he had experienced a mild coccygeal trauma while playing soccer. Seven days later he presented paresthesia of the lower limbs and, less than 24 hours later, bilateral hypoesthesia and paraparesis. He was unable to initiate urination or defecation, but was not incontinent. He denied fever and any infectious episodes over the previous weeks.\nThe patient had been diagnosed of Blau syndrome at the age of 5. The condition manifested as a generalized papulous rash, recurrent arthritis, and tenosynovitis, which started when he was 2 years old. His mother had been misdiagnosed as having rheumatoid arthritis as a child, after presenting similar symptoms. Genetic study confirmed an autosomal dominant mutation in the NOD2/CARD15 gene. The patient had been treated earlier with corticosteroids and methotrexate and, over the previous 8 years, since the diagnosis, had also received etanercept, with good disease control. He had never presented ocular manifestations.\nPhysical examination revealed a normal mental status, with no cranial nerve involvement. Funduscopic examination was normal. Muscle tone strength and deep tendon reflexes of the upper limbs were normal. He had hyperreflexia in both lower limbs, an extensor plantar reflex and bilateral exhaustible clonus. Muscle strength in the lower limbs was decreased, graded 2 to 4 out of a maximum of 5 in the different muscle groups, the most highly affected being the psoas and quadriceps. He had tactile and pain hypoesthesia with a sensitive level at T12 and normal thermal and vibratory sensation. Painless camptodactyly and flexion contractures of the proximal interphalangeal joints of the fourth and fifth fingers had already been documented, and there were no inflamed joints. He had no spleen or liver enlargement and no acute skin lesions. The remainder of the examination was normal.\nBlood analyses were unremarkable, except for a high erythrocyte sedimentation rate (85 mm/h, normal value <10 mm/h). Cerebrospinal fluid glucose level was normal, protein was slightly elevated (78 mg/dL, normal value 15–45 mg/dL), and IgG level was high (7.4 mg/dL, normal value <3.4 mg/dL), without pleocytosis or oligoclonal bands. Bacterial, viral, and fungal microbiological tests were negative, including mycobacteria.\nCranial magnetic resonance imaging (MRI) study was normal. MRI of the spinal cord revealed bifocal high-intensity white matter lesions on T2-weighted images (T2WI), one extending from the second to the fourth cervical (C2–C4) level and another at the conus medullaris (T12-L1), which enhanced after contrast administration (Figures and ). Diffusion-weighted imaging (DWI) and apparent diffusion coefficient maps failed to show water movement restriction.\nConsidering the previous reports of similar adverse reactions with anti-TNF-α [–], etanercept was discontinued. The patient received intravenous methylprednisolone pulse therapy (1 g/day) during 5 days, followed by oral prednisone starting at 1 mg/kg, with slow dose tapering for 6 weeks. Clinical improvement was noted 36 hours after the first infusion, and the patient was able to walk without help and regain sphincter control 5 days later. Mild proximal muscle weakness, hyperreflexia, and paresthesia persisted during the next 2 weeks, with progressive amelioration. Four weeks later, his recovery was nearly complete, with slight hyperreflexia in the lower limbs.\nFollow-up MRI performed 6 weeks after the first study revealed complete resolution of the cervical cord lesion and an improvement with no enhancement of the conus medullaris lesion ().\nAt his last clinical follow-up visit, 3 months after onset, the patient was completely symptom-free, and the neurological examination was normal. His baseline treatment was switched to corticosteroids and methotrexate. New biological drugs will be reconsidered depending on his disease control, but anti-TNF-α agents are excluded. |
A 73-year-old man had a past operative history of ruptured colonic diverticulitis treated by Hartmann’s procedure in 2007, and perforated peptic ulcer treated by pyloroplasty in 2009. He suffered from abdominal and tarry stool for 5 days in March 2011, so he was admitted to a rural hospital for further survey and medication. An initial upper GI endoscopy showed gastric ulcer only, but hypotension with tachycardia and a drop in hemoglobin of 9 g/dl from 12 g/dl occurred the next day. Intravenous fluid and blood transfusion with packed red blood cell were prescribed. Abdominal aortic aneurysm (AAA) with rupture was highly suspected, so CTA of aorta (Fig. ) was carried out which disclosed an AAA with swollen bowel loop. He was transferred to our emergency department (ED) for consideration of vascular surgical intervention.\nOn arrival in our ED, vital signs were a respiratory rate of 16 breaths per min, a heart rate of 130 beats per min, a blood pressure of 98/73 mmHg, and a body temperature of 35.6 °C. Physical examination revealed a pale conjunctiva, no heart murmur, clear breathing sound, old operative scar over middle abdomen, and hypoactive bowel sound with tenderness over the periumbilical region. Leukocytosis, anemia, abnormal liver profiles, and metabolic acidosis were found. Laboratory investigations were summarized in Table .\nA cardiovascular surgeon carried out emergent angiography of aorta, which depicted bleeding from the wall of the AAA and chronic total occlusion of the left external iliac artery, so a modified aorto-uni-iliac stent graft (Fig. ) was implanted with femoral-to-femoral bypass, which underwent smoothly. A general surgeon performed exploratory laparotomy and found a massive blood clot with a volume of about 1310 ml filling the entire lumen of the small intestine, a dilated and swollen duodenum about 6 cm in diameter with ecchymosis, and a fistula about 2x2cm over the third portion of the duodenum (Fig. ). Duodenostomy with primary closure for repair of fistula defect, partition of the duodenum between the 2nd and 3rd portions, and side-to-side gastrojejunostomy were completed smoothly. He was admitted to the surgical intensive care unit for postoperative care. CTA of aorta showed neither endoleakage nor intraabdominal abscess on postoperative day 17. He was discharged without complications and returned for follow up at the out-of-patient department on postoperative day 25. |
The patient was a 28 year-old Hispanic man with a history of end stage renal disease due to hypertensive disease since the age of 10 years, complicated by malignant hypertension. His surgical history was remarkable for a remotely failed renal transplant requiring an allograft nephrectomy. He had been on hemodialysis for about a decade since, and transitioned to peritoneal dialysis just six months prior to presenting to our facility with sepsis due to new-onset peritonitis.\nIn the outpatient clinic, the patient had been diagnosed with external cheek cellulitis that responded to a short course of empiric cephalexin. He overcame this only to experience three days of abdominal pain near his dialysis catheter. The pain was constant and severe pain, worsened by draining and filling during dialysis sessions. He denied any fevers, chills, nausea, vomiting, diarrhea, oropharyngeal and genitourinary complaints or decrease in recent ultrafiltration. Exam was remarkable for a blood pressure of 167/99 mmHg, heart rate of 107 beats per minute and abdominal tenderness to palpation diffusely with the catheter exit site clear dry and intact without exudate or erythema.\nHe quickly underwent peritoneal fluid examination, revealing an absolute neutrophil count of 14,458 cells/mm, confirming the diagnosis of bacterial peritonitis. His gram stain revealed gram-negative diplococci and subsequent chocolate agar plated cultures grew Neisseria species, not gonorrhoeae or meningitidis. Further biochemical speciation (via RapID NH System by Remel, Thermo Fisher Scientific Inc., Waltham, USA) showed Neisseria mucosa to be the etiology of the patient’s bacterial peritonitis. Given this is typically nonpathogenic, further susceptibility was not performed.\nThe patient was placed on empiric intraperitoneal vancomycin 1 gram and cefepime 1 gram therapy and later transitioned to oral ciprofloxacin 500 mg daily for two weeks. During the hospitalization, repeat peritoneal fluid analysis confirmed a resolving leukocytosis and bacterial cultures without growth. Subsequently, the intraperitoneal catheter was preserved without recurrence of peritonitis to date. |
The 41-year old woman traveled to Kenya and Tanzania between 23 July and 5 August, 2017. She left China on 22 July and arrived in Nairobi, Kenya on 23 July. Her route in Kenya and Tanzania is shown in Fig. . She returned to China, landing at Guangzhou Airport on 6 August. Before leaving for Africa she received a yellow fever vaccination and was given advice on anti-mosquito measures to prevent malaria at a local international travel health care center.\nThe patient visited the Serengeti National Park in Tanzania and the Masai Mara National Reserve in Kenya between 28 July and 2 August. She was bitten by an insect identified by the local driver as a tsetse fly while taking animal photographs on 29 July at the Serengeti National Park. Her husband and the local driver were bitten by the same species of insect at the same time.\nOn 8 August, 2017, 2 days after she returned to China, she developed a fever with a temperature of 40.1 °C along with symptoms of dizziness, fatigue and rigors. She sought medical assistance at a local hospital where she was initially treated with intravenous fluids and broad-spectrum antibiotics. The following day, she was transferred to the fever department at the Fujian Medical University Union Hospital where she was examined by another physician. Clinical evaluations at the time showed that the patient was alert and well orientated, with no lymph-node enlargement or any rashes noted. The most striking abnormality was the presence of a red chancre measuring 22 mm in diameter on her right heel (see Fig. ).\nDespite the anti-infection treatment she was given, the patient remained febrile until 11 August, when she was transferred to the Infectious Diseases Department of the same hospital. At this time, her condition worsened with persistent fevers, headache, productive cough, and worsening jaundice.\nHer blood tests on 14 August showed liver dysfunction (glutamic pyruvic transaminase [ALT]: 212.0 IU/L [0–40 IU/L], glutamic-oxaloacetic transaminase [AST]: 168.0 IU/L [0–46 IU/L], alkaline phosphatase: 460.0 IU/L [3–104 IU/L]). In addition she was noted to have hyponatremia (131.2 mmol/L [135.0–148.0 mmol/L]), hypokalemia (3.11 [3.5–5.5 mmol/L]), and thrombocytopenia (70 × 109/L [[100–300] × 109/L]). A computed tomography (CT) scan of her chest showed a strip shadow in both lungs, which the doctor thought could be pneumonia.\nOn 14 August, the patient’s blood sample was sent to the Fujian Provincial Center for Disease Control and Prevention to investigate the presence of Plasmodium species. The giemsa-stained thin and thick blood smears as well as the malaria antigen test (immuno chromatographic test) were both negative for the Plasmodium species. However, a few trypanosomes were found in both the thin and thick blood smears. Blood examination confirmed a high parasitemia, with one to two trypanosomes seen every five fields under microscopy at 1000× magnification (see Fig. and Additional file 2: Video 1).\nAs the presence of trypanosomes in the blood was confirmed, a normal cerebrospinal fluid (CSF) puncture performed on the same day revealed no trypanosome with two white blood cells (WBCs)/mm3, which signified first-stage disease.\nTo confirm the diagnosis of rhodesiense HAT on a molecular level, polymerase chain reaction (PCR) techniques were used. Nucleic acid DNA was extracted from the patient’s peripheral blood using the DNeasy® Blood & Tissue Kit (QIAGEN, Germany). Two specific genes namely T. b. rhodesiense-specific human serum resistance--associated (SRA) gene and the Trypanosoma spp. universal internal transcribed spacer (ITS) gene, were targeted by the PCR method, as previously described [, ]. After PCR products were sequenced both for SRA and ITS genes, it was found that 284 bp of the SRA gene sequence obtained match 100% to the T. b. rhodesiense partial SRA genes (GenBank accession numbers: Z37159, AJ345058 and AJ345057), and 450 bp of the ITS gene sequence was 97% similar to T. brucei isolate ITS gene (GenBank accession numbers: JX910373 and AF306771).\nSince no suramin was available immediately after diagnosis, 200 mg pentamidine was initially given by intravenous (IV) injection on 15 August, and the same dose was then injected intramuscularly on 16 August and 17 August. On 16 August, after the second dose of pentamidine, no trypomastigotes were detected in thin and thick blood films. After three doses of pentamidine, the patient’s condition rapidly improved, her fever disappeared, the chancre reduced in size and the cough lessened, although her headache persisted. The liver function tests and electrolytes improved, but the platelet counts increased rapidly, reaching a level of 374 × 109/L ([100–300] × 109/L).\nSuramin was available on 18 August provided by the World Health Organization (WHO). Pentamidine was discontinued and suramin was given at a test dose of 200 mg by IV injection on 18 August, and the treatment dose was then escalated to 1 g on days 3, 7, 14, 21 and 28. No adverse reactions were observed during treatment. After three doses of 1 g suramin, the patient’s liver function indices and electrolytes normalized. The platelet index, however, continued to rise, peaking at 588 × 109/L after the second 1 g dose of suramin, but with the third dose, the platelet index began to decrease (434 × 109/L). At this time, the patient felt much better, but continued to have a mild headache and cough. A new CSF analysis performed on day 13 (30 August) post-suramin initiation revealed an absence of trypanosomes and 2 WBC/mm3.\nThe patient was asymptomatic except for an occasional headache 1 month after the suramin treatment commenced. All blood test alterations had normalized at this point. |
This is a case of a 30 year-old gentleman with a history of Crohn's disease. He was on a regimen of infliximab, infused every 8 weeks and oral methotrexate daily. He had no other significant medical history. He was in his usual state of health until he developed a sore throat and fevers on day 0. His symptoms began while he was traveling in Europe. During his trip, he took a 10-day course of amoxicillin and felt some improvement.\nAfter returning to the US he felt well but on day 14 he developed fevers and sore throat again. He was evaluated at an urgent care clinic and sent home with a diagnosis of a viral syndrome and instructed to treat this with non-steroidal anti-inflammatory agents. He felt some improvement initially but presented again on the day of admission with concern that he may not be well enough for his infusion of infliximab. He was found to have a temperature of 38.3 C and on day 30 was sent to the emergency department for further evaluation.\nIn the emergency department, he reported a non-productive cough, fevers, and sore throat. He had elevated liver enzymes: aspartate aminotransferase (AST) 340, alanine aminotransferase (ALT) 540, alkaline phosphatase 145. Additional testing was sent including a Monospot test, cytomegalovirus (CMV) and Epstein's Barr virus (EBV) serum viral levels, respiratory viral panel by PCR, adenovirus serum viral level, HIV antibody/antigen as well as HIV viral level, viral hepatitis serologic panel, human herpesvirus type 6 (HHV6) serum viral level, Varicella zoster virus (VZV) serum viral level, and syphilis IgG. He was admitted to the inpatient medicine service for fevers and hepatitis of unknown origin. His social history was remarkable for frequent trips to the Midwest for work. He did not pursue outdoors activity while traveling. He did not smoke, drink alcohol or use illicit drugs.\nOn day 31 he developed daily fevers to 40 C and subsequently progressed to hypoxemic respiratory failure that required high-flow supplemental oxygen and transfer to the intensive care unit. He had a chest CT showing ground-glass opacities at the lung bases and a left upper lobe nodular opacity (, ). His CT also demonstrated tonsillar enlargement and splenomegaly. He had a laryngoscopy performed that revealed an exudative pharyngitis. He was noted to have atypical lymphocytosis that peaked to 12,000 cells/uL on day 33. He was started on broad-spectrum antibiotics for presumed hospital-acquired pneumonia; including coverage of atypical organisms. He underwent a bronchoscopy on day 33. Bronchoalveolar lavage (BAL) fluid was negative for Legionella culture, AFB stain and culture, pneumocystis stain, and bacterial culture. Mycoplasma, zygomycetes and Mycobacterium tuberculosis were not detected by PCR. A respiratory viral panel by PCR from the lavage fluid was positive for Bocavirus and one of 2 samples for aspergillus galactomannan was moderately elevated but later determined to be negative on repeat testing.\nOn day 33 his AST peaked at 351 and his ALT at 662. His viral studies sent earlier returned negative. He had further serologic testing for atypical organisms including Blastomyces, Coccidioides, Q fever, Bordetella pertussis, as well as urine Legionella antigen that all returned negative. He was started on methylprednisolone and his fevers improved. His antibiotics were discontinued when his bacterial cultures were negative at 48 h. On day 37 the fungal PCR from BAL returned positive for Histoplasma capsulatum and cultures from that fluid later grew the organism. Urine Histoplasma antigen testing, sent earlier during his hospitalization, ultimately returned with a titer of 2.16 ng/mL (normal high 0.1 ng/mL). His Coccidioides antibody was also positive, but this was felt to be due to cross-reactivity from his Histoplasma infection.\nOn day 37 he was started on liposomal amphotericin B and 6 h later he had his highest fever to 42 C along with hypotension, tachycardia and worsening hypoxemia despite having received a dose of methylprednisolone earlier that day. He was felt to have a paradoxical worsening with treatment and exacerbation of immune reconstitution inflammatory state rather than an infusion reaction due to amphotericin given this time course . His infliximab was restarted the following day with subsequent resolution of his fever, hemodynamic instability and improvement in his respiratory status within hours of this infusion. He continued amphotericin B for one week before transitioning to oral itraconazole. His ALT/AST improved significantly. He was discharged on oral itraconazole with continued clinical improvement and recovery as an outpatient. |
A 67-year-old woman presented to the emergency department at our university hospital complaining of constipation and right flank pain. She suffered from uncontrolled hypertension and type 2 diabetes mellitus. Additionally, the patient claimed that she was diagnosed with an adnexal cyst 7 years ago. Her previous gynecologist did not manage to determine exactly whether the cyst was ovarian or abdominal in origin and no further surgical interventions or sonographic imaging follow-up were carried out. On presentation, the patient was hypertensive and tachycardic. She was afebrile and her oxygen saturation was normal. Upon clinical examination, her abdomen was distended and non-tender. Her cardiac auscultation revealed arrhythmic arrhythmia. The laboratory tests were unremarkable except for a raised blood glucose level of 201 mg/dL. Her complete blood count, electrolytes, liver and kidney function tests were within the normal limits. A 12-leads electrocardiogram revealed atrial fibrillation with rapid ventricular response. A computed tomography scan (CT scan) of the abdomen revealed the presence of a large multilocular cystic mass measuring 17 X 26 cm in its anterior-posterior and transverse diameters respectively. The cyst occupied most of the abdomen and caused bowel obstruction and right sided hydronephrosis (Fig. ).\nThe initial differential diagnosis was either an omental cyst or abdominal abscess. The patient went preoperative optimization regarding the hypertension, hyperglycemia, and atrial fibrillation. The patient underwent a midline laparotomy. A large abdominal cyst adhered to the greater omentum was found. The cyst received its extensive vascular blood supply form the greater omentum (Fig. a). The uterus and both ovaries were unremarkable and were separate from the cyst. After ligating the cystic feeding vessels, an en-bloc cystectomy was performed successfully (Fig. b). Gross examination of the resected specimen revealed a large cystic mass measuring 45 cm in its longitudinal diameter and weighting 4.5 Kilograms. The cyst was multilocular and was filled primarily with clear fluid. Additionally, some cystic cavities were hemorrhagic.\nMicroscopic examination of the cyst revealed a columnar endometrial lining with underlying endometrial stroma and siderophages (Fig. ). Based on the cyst’s characteristics, an abdominal endometrioma was diagnosed. The patient was hospitalized for 4 days and her postoperative recovery period was uneventful. |
A 19-year-old female patient reported to our Department with the chief complaint of swollen lower lip since 1 year. Her medical history revealed history of surgical treatment of a lump in the breast 2 years back. History also revealed presence of dysmenorrhea, frequent diarrhea and generalized body ache since 3 years. Her parents and other siblings were normal. On general examination, the patient appeared thin built. Her left palm and left thumb showed presence of lobulated nodules [].\nExtra-oral examination revealed presence of multiple skin colored flat papules (trichilemmomas) on her face involving the bridge of the nose, forehead region and on the upper lip measuring around 1-5 mm in diameter []. Lower lip also showed multiple non-tender papular growths around 1-3mm in diameter, clustered at certain places [].\nIntra oral examination revealed smooth whitish papillomatous lesions involving the dorsal surface of the tongue and the buccal mucosa measuring 1-5 mm in diameter [Figures and ]. On the basis of history and clinical examination, the patient was provisionally diagnosed as a case of CS.\nSeveral investigations were performed for this patient to rule out other hamartomas and malignancies that included complete blood investigations, thyroid profile and thyroid scan, ultrasonography of breast, thyroid, ovaries and nodules of palm, mammography, orthopantomogram, Magnetic Resonance Imaging of brain, Digestive endoscopy and Fine needle aspiration cytology from the lesions of the breast and thyroid along with incisional biopsy from the tongue and the lip lesions.\nHematological investigations of the patient and the thyroid profile (T3, T4. Thyroid stimulating hormone did not reveal any changes from the normal reference values. The mammography and orthopantomogram of the patient revealed normal findings. MRI of brain did not reveal any lesions in the cerebellum. The ultrasonographic examination of the thyroid gland showed multiple nodules in the both lobes of the thyroid gland, which were mostly echogenic. Few cysts were also seen within the thyroid with internal finger like solid projections showing increased vascularity (intracystic papillary mass) which gave the impression of multinodular goiter []. Similarly, the Tc-99 m pertechnate thyroid scintigraphy scans were suggestive of euthyroid multinodular goiter with a dominant cold nodule present at the lower pole of the right lobe of the thyroid gland. The FNAC from the thyroid gland yielded blood mixed aspirate and showed occasional groups of follicular epithelial cells in the background of abundant colloid giving impression of multiple colloid nodules in the thyroid gland.\nThe Ultrasonography examination of breast showed multiple nodular cystic areas bilaterally suggestive of fibroadenomas which were confirmed by FNAC and miscroscopy while a cystic area with nodular solid component eccentrically originating from the walls occupying more than 50% of the cystic lumen was seen at a single area in the left breast which was suggestive of intracystic complex mass [ and ]. Ultrasonography of the left palm showed 12 mm × 9 mm sized mildly hypoechoic lobulated nodule with internal arterial and venous vascular channels suggestive of arterio-venous malformation.\nThe digestive endoscopy of the stomach and the duodenum revealed multiple polypoid lesions having smooth overlying mucosa. Some of them were hyperplasic; some were sessile while others were adenomatous, fibroid like. These were randomly distributed throughout the stomach and duodenum, mostly measuring less than 1 cm in diameter [].\nHistopathology from the sections taken from the lower lip and tongue revealed hyperkeratinized stratified squamous hyperplastic epithelium with no inflammatory evidence in the connective tissue suggestive of epithelial hyperplasia [].\nMultidisciplinary approach was followed and various opinions were taken from gynecologists, gastroenterologist, general surgeon and oral pathologist. Patient is under regular follow-up and care. |
A 36-year-old man presented to the general surgery clinic of our hospital complaining of abdominal pain for 13 d.\nThe patient’s symptoms started 13 d ago and were characterized by persistent dull pain in the upper abdomen; the symptoms had been relieved after symptomatic treatment in a local hospital. However, abdominal pain occurred again 1 d ago, and the symptoms were the same as previously experienced.\nThe patient had no previous medical history.\nThe patient’s temperature was 36.8 °C, heart rate was 87 bpm, respiratory rate was 18 breaths per minute and blood pressure was 122/76 mmHg. Anemia signs and slight tenderness in the upper abdomen were observed, and there were no other typical pathological signs. Our first clinical considerations for the abdominal pain were as follows: Space-occupying lesions of the small intestine (possible stromal tumor), followed by possible choledocholithiasis.\nBlood analysis showed a hemoglobin level of 75 g/L (normal range > 120 g/L) with a normal leukocytosis level and platelet count. The prothrombin and partial thromboplastin times, the serum C-reactive protein level and the blood biochemistry and urine analyses were normal. The electrocardiogram and chest x-ray results were also normal.\nMagnetic resonance cholangiopancreatography (MRCP) revealed an abnormal mass signal in the distal duodenum with proximal intussusception (Figure ). An abdominal enhanced computed tomography (CT) scan suggested gallstones in the lower part of the common bile duct, mild dilatation of the main pancreatic duct, a slightly disordered horizontal structure of the duodenum and a suspicious space occupying the ascending part of the duodenum with proximal duodenal intussusception (Figure ). A space occupying stromal tumor was considered, and the tumor boundary was clear (Figure ). Upper gastrointestinal radiography revealed that the duodenal bulb was adequately filled, the descending part of the duodenum was tortuous and long, the distal end was filled and passed well and there were no signs of obstruction. Gastroscopy revealed a 0.6 cm × 0.5 cm ulcer in the anterior wall of the duodenal bulb, and no abnormality was found in the descending part of the duodenum. |
A 68-year-old asthmatic male patient presented to our center with 12 days history of melena. He denied any previous episode of melena or hematochezia or bleeding from another site. The patient did not have any other associated symptom, and had no other co-morbidities, or medication use. Upon referral, he was uncomfortable and looked pale. He was vitally unstable, with a blood pressure of 90/60 mmHg and a pulse rate of 120 beats/min. The examination revealed the presence of clotted blood on the anal verge, and some tarry stool on digital rectal examination. The hemoglobin level was 7.7 g/dl, the hematocrit was 22.8, and the blood urea nitrogen was 8 mg/dl. The prothrombin time and the partial thromboplastin time were normal.\nResuscitation was performed with transfusion of 2 units of packed red blood cells and intravenous fluids. He was admitted to the ICU for intensive monitoring. After admission and stabilization, upper and lower endoscopies were performed without demonstrating the bleeding site. They only revealed clotted and red blood throughout the colon.\nTechnetium-labeled red blood cell bleeding scan was done to localize the site of bleeding. This scan showed no evidence of early focal increased uptake in the abdomen to indicate active gastrointestinal bleeding during early images, but in the delayed images, it revealed that there was a focal uptake in the right and transverse colon. After that, capsule endoscopy was also performed without findings. As the angiography became available, the patient underwent selective angiography without findings noted at that time. These tests were inconclusive because they were performed while the episodes of bleeding ceased.\nAfter 8 days of conservative management and negative investigations to define the cause of the bleeding, a sudden drop in hemoglobin level from 10.8 mg/dl to 6.9 mg/dl occurred over 12 h, which mandated operative management. Exploratory laparotomy was performed. Extensive jejunal saccular pouches were found 10 cm distal to duodenojejunal junction extending 1.6 m distally Fig. . The bleeding was difficult to control and the decision to clamp the major branched was performed. Division of the small bowel proximal and distal to the diseased part using gastrointestinal stapler was performed with side to side primary anastomosis Fig. . The specimen was a part of small bowel, 117 cm in length, with congested wall and multiple pouches at the mesenteric site. Opening of the specimen showed normally looking mucosa with active bleeding that stopped after awhile. No polyps or masses were detected. We reviewed the angiography achieve after that and a suspicious shadow reflecting the diverticular outpouching was detected.\nMicroscopic examination of the specimen revealed many diverticula; some of which being true diverticula, while the others are devoid of muscularis propria (false diverticulae). Within the diverticula and in the intervening portions of the bowel wall, there were numerous dilated thick- and thin-walled small blood vessels in the submucosa. Additionally, submucosal intermediate-sized vascular clusters and feeder vessels in the muscularis propria and serosa were present. The overall features were those of small intestinal diverticulosis and arteriovenous malformations. The latter involves the diverticula and intervening portions of the bowel wall Fig. .\nPostoperatively, the patient was doing well, discharged home on day 5 postoperatively, with an uneventful postoperative course. He was followed up 2 years after that without complications. |
A 36-year-old Caucasian male presented to the emergency room complaining of a one-day history of abdominal pain. His main symptoms were that of nausea and vomiting, but he also reported periods of diarrhea. In the emergency room, his initial evaluation was significant for lab studies demonstrating a mild metabolic acidosis with a bicarb of 16.6 mEq/L, an elevation of his creatinine to 1.93 mg/dL, and a serum lactate level of 5.4 mmol/L. A computed tomography (CT) scan of the abdomen and pelvis was obtained and reviewed. The appendix was thought to be normal. There was no evidence of free intraperitoneal air, abscess, or volvulus. There did appear to be evidence of enterocolitis involving the ilium, cecum, and the proximal ascending colon. There was no evidence of pneumatosis or obstruction. Over the course of 12 hours the patient underwent conservative medical management, which included intravenous hydration, intravenous steroids, Toradol and Dilaudid for pain management, and the occasional dose of Ativan for agitation. Failure of conservative management along with medical decline lead to an exploratory laparotomy with a right hemicolectomy for an ileocolic intestinal infarction. Postoperative repeat CT scan of the abdomen incidentally showed pulmonary nodules in the lower lobe. A CT scan of the chest was then obtained, which revealed air in the left chest wall and axilla (Figure ). On physical exam there was very subtle mottling of the left shoulder. The patient was emergently taken back to the OR for further exploration of the shoulder with excisional debridement. Seropurulent fluid and extensive subcutaneous emphysema along the fascial planes of the left chest wall were consistent with necrotizing fasciitis. Both wounds were packed with a gauze bandage roll soaked in saline and then the patient was transferred back to the intensive care unit.\nOver the course of 15 days the patient underwent repeat trips to the OR for re-exploration, incision and drainage of the wounds, and dressing changes (Table ). On postoperative day 17, the wound, ostomy, continence nurse (WOCN) was consulted to evaluate the wound and make treatment recommendations.\nOn exam, there was still a significant amount of nonviable tissue in the base of the left chest and axilla wound. The left chest wound measured 8 x 19 x 8 cm and the left axilla wound measured 6.5 x 25 x 4 cm. NPWTi-d with ROCF-CC was applied to these wounds to aid in the removal of the nonviable tissue. Figures - show the presentation of the wounds prior to the application of the NPWTi-d with ROCF-CC.\nDuring the application of the NPWTi-d with ROCF-CC, care was taken to ensure that the contact layer of the dressing was in contact with the entire wound base. Barrier rings were applied to the skin creases along with any area that presented as a high risk for inadequate seal of the drape (Figure ). The therapy settings for the left axilla were 85 ml of hypochlorous acid solution (Vashe®, SteadMed, Fort Worth, TX), dwell time of 10 minutes with NPWT every hour at -125 mmHg. Therapy settings for the left chest wound were 50 ml of hypochlorous acid solution, dwell time of 10 minutes with NPWT every hour at -125 mmHg.\nDuring the first dressing change after the application of the NPWTi-d with ROCF-CC, there were notable improvements in the quality of the tissue in the wound bed (Figure ). The left chest wound bed was noted to have 100% red healthy tissue and the left axilla wound bed had a decrease in nonviable tissue. There was, however, seropurulent drainage noted deep within the intramuscular structures of the left axilla wound.\nNPWTi-d with ROCF-CC was reapplied to the axilla wound ensuring that the contact layer was placed deep into the wound bed to facilitate the removal of the seropurulent drainage. The left chest wound was transitioned to NPWTi-d to continue to promote granulation tissue (Figure ). The instillation solution was switched to normal saline due to blockage alarms we encountered with the hypochlorous acid solution without resolution.\nThe current treatment regimen was continued for two days until the next dressing change. At that time both wound bases were 100% red with granulation tissue present (Figure ). The seropurulent drainage deep in the intramuscular structures was no longer present. Since there was adequate removal of nonviable tissue and the quality of the tissue had significantly improved, the WOCN made the decision to transition both wounds to NPWTi-d instilling normal saline solution with a dwell time of 10 minutes and NPWT every two hours at -125 mmHg.\nFor the next two weeks the treatment regimen utilizing NPWTi-d to the wounds continued. The dressings were changed three times a week at the bedside by the WOCN. During initial assessment, the axilla wound measured in total volume 650 cm³ and the chest wound measured 1,216 cm³. After 17 days utilizing the combination of NPWTi-d with and without ROCF-CC, the axilla wound measured in total volume 342.25 cm³ and the chest wound measured 554.4 cm³. This presents a significant decrease in overall measurements. At this time, NPWTi-d was discontinued and standard NPWT with black granufoam was utilized to both wounds at -125 mmHg (Figures -).\nNPWT was utilized for approximately a month to promote granulation tissue and assist with wound contraction (Figures -). Once the wound had made vast improvements, NPWT was discontinued and a non-adherent antimicrobial alginate dressing (Silvercel™ Non-adherent, Acelity, San Antonio, TX) was lightly packed into the wound bed and covered with a dry sterile dressing. This dressing was then changed three times a week by a visiting nurse association. The wound measurements prior to discharge showed significant improvement in comparison to the wound measurement at presentation, with the left axilla wound measuring 1.5 x 10.5 x 0.3 cm and the left chest wound measuring 3.5 x 9 x 5 cm. |
Our patient was a 64-year-old gentleman who complained of nocturia, diurnal frequency, poor flow and terminal dribbling. This was associated with and intermittent left scrotal swelling. He had a medical history of late-onset asthma, erectile dysfunction and hypertension.\nWhen seen at clinic, he was found to have a left scrotal swelling that extended into the inguinal region associated with a cough impulse. It was felt that this was either a hydrocele or a sliding hernia and an USS was arranged. His uroflometry showed that he had an obstructed flow, but he emptied his bladder on post-residual scanning. In addition to the USS, he was commenced on an alpha-blocker for bladder outflow obstruction and a flexible cystoscopy was arranged.\nHis USS diagnosed an inguinal hernia containing omental fat. At this point a referral to a general surgeon was made. His cystoscopy showed a normal urethra with a small, short prostate and a high bladder neck. The bladder itself was unusual. Upon filling a large left-sided bladder, diverticulum was seen.\nBased on this cystoscopic finding, a micturating cytogram was arranged to evaluate this diverticulum as seen in the figures provided (Figures and ). This showed an unusual huge bladder diverticulum that had herniated through his inguinal canal, filling his left hemiscrotum.\nThis was obviously contributing to his urinary symptoms by having a reservoir that could not be emptied on micturition. It was felt that this could deteriorate, and surgical excision was agreed with the patient.\nOur aim was to perform a diverticulectomy with repair of the hernia defect. For this we used a retropubic lower midline incision. Dissection was made down onto the bladder until the diverticulum was identified. Dissection was attempted to remove the diverticulum off the outer bladder surface and spermatic cord as it entered the deep inguinal ring. Unfortunately, the diverticulum was adherent to adjacent structures and this was not possible.\nTo confidently identify the neck of the diverticulum, the bladder was therefore opened and held with stay sutures. This allowed dissection of the adhesions between the outer surface of the bladder and the diverticulum. However, it was not possible to identify the spermatic cord at this stage; therefore, the neck of the diverticulum was transected in order that a point of traction was created.\nThis facilitated dissection with a combination of blunt and sharp technique. The spermatic cord was identified and safely separated from the hernial sac including the diverticulum. The plane was followed into the scrotum where the entire diverticulum was removed from the hemiscrotum. There were no adhesions within the scrotum of the diverticulum to surrounding layers, which made a secondary scrotal incision unnecessary. Haemostasis was ensured in these areas and the bladder was closed with two layers of continuous vicryl.\nTwo weeks following the procedure he underwent a trial of voiding, which he passed successfully. He was discharged home and returned 6 months later with for a follow-up ultrasound of his renal tract, which revealed no further pathology. |
A 9-year-old female child presented with an asymptomatic slowly progressive palatal mass for 1 year (usually noted in the superficial lobe of parotid in the mid-adult years). There was no history of dysphagia, odynophagia, sleep apnea, voice change, weight loss, loss of appetite, and fever. The clinical examination revealed a firm, nontender, nonpulsatile, round, bluish pink mass originating from the left side of hard palate crossing midline (the lesion was firm in our case, whereas it is usually freely movable if present at any other site in the oral cavity). No ulceration was noted on the surface of swelling . The computed tomography (CT) scan showed well-defined heterogeneous mildly enhancing soft-tissue mass arising from hard palate (measuring 4.4 cm × 3.4 cm × 2.2 cm), causing significant bulge in oral cavity with no obvious involvement of soft palate and no bony erosion. There was slight calcification in the center of mass, but no cystic component or fat tissue component within the mass. Based on CT findings, it was diagnosed with a benign palatal mass. The surgical excision of the benign palatal tumor was planned out. Preoperative planning involved replication of tumor in cast []; impression made using medium-body putty material. Tumor impression replicated in the cast was trimmed, following which obturator was fabricated []. The patient was operated through transoral approach under general anesthesia. The mass was totally removed [Figures and ] with submucosal dissection and histological diagnosis was confirmed. Immediately, after the removal of palatal tumor, the obturator was given for 2 weeks []. The obturator was removed after 15 days of the surgical procedure. The patient was recalled after every 15 days for the next 2 months, to assess the presence of any secondary infectioninfection; is showing the progress after 3 weeks. The patient was kept under observation for the next 6 months.\nThe tumor is composed of exclusively of neoplastic myoepithelial cells. The tumor shows lobules and sheets of proliferating tumor cells, which are predominantly plasmacytoid []. Tumor cells are round cells with eccentric nuclei with eosinophilic often hyaline appearing cytoplasm giving it a plasmacytoid appearance [] and these cells are often referred to as “Hyaline Cells.” Little intercellular fibrous stroma is present.\nMyoepithelial differentiation of tumor cells is confirmed with immunohistochemistry using markers cytokeratin – CK-5 and CK-6, embryonic membrane antigen (EMA) [], and smooth muscle actin (SMA) []. SMA shows focal positivity in tumor cells. EMA shows positivity in salivary ducts and is negative in tumor cells. Cytokeratin shows positivity in tumor cells that is CK-5 [] and CK-6 [] shows diffuse positivity in tumor cells. |
We describe a 54-year-old woman who initially presented in 1999 at the age of 40 with newly diagnosed CML identified on routine blood work during pregnancy. She eventually miscarried. She was initially treated with Hydrea and underwent sibling allogeneic HSCT with her brother as a donor in January 2000. She tolerated the transplant without complications and was in remission until January 2012 when routine blood work identified increased serum protein and anemia. Serum protein electrophoresis (SPEP) demonstrated an abnormal band in the gamma region and M protein spike of 1.9. Urine protein electrophoresis (UPEP) demonstrated a monoclonal kappa light chain. Bone marrow biopsy demonstrated a monoclonal plasmacytosis of 10–12% consistent with plasma cell dyscrasia. There was no evidence of CML. FISH studies and BCR-ABL testing were negative. A bone study was negative for blastic and lytic lesions. Cytogenetics showed a normal male karyotype of donor origin. These findings were consistent with a new diagnosis of IgG kappa smoldering multiple myeloma.\nOur patient was followed with watchful waiting until repeat bone marrow biopsy showed multiple myeloma with rapid progression to active disease. FISH study at diagnosis was positive for an IgH/FGFR3 rearrangement. She was treated with Revlimid, Velcade, and dexamethasone beginning in February 2013. She then continued to autologous HSCT with melphalan as a conditioning regimen in July 2013.\nBased on the patient's full chimerism, it was recommended that her sibling donor be evaluated. His labs were significant for an elevated serum protein, SPEP with an abnormal band in the gamma region, and an M protein spike of 2.3. Her sibling donor underwent bone marrow biopsy in April 2012 that showed monoclonal plasmacytosis (~20% on CD138 stain) consistent with myeloma. Similar to our patient, his FISH studies were also positive for IgH/FGFR3 rearrangement in 5.5% of cells. Flow cytometric analyses also showed a monoclonal plasma cell population with cytoplasmic kappa light chain restriction. Findings were consistent with IgG kappa smoldering multiple myeloma that also progressed to active disease. He was also treated with Velcade, dexamethasone, and Revlimid before undergoing autologous stem cell transplant in February 2014. |
A 91-year-old Korean female with a history of hypertension and cerebral infarction presented with an asymptomatic, well-defined, solitary, 3.4-cm-sized, reddish, hard, palpable protruding mass with central crust on the lateral aspect of the right upper eyelid that had been present for 6 months and had increased in size rapidly during the past 3 months ().\nTwo punch biopsies at the central and lateral part of the mass were performed. Hematoxylin and eosin staining showed storiform cellular infiltrate of pleomorphic spindle and polygonal cells with frequent atypical mitoses, and so the lesion was suggested to be AFX. Computed tomography performed to evaluate invasion levels showed skin and subcutaneous fat as heterogeneous enhancing mass on the lateral aspect of the right upper eyelid without infiltration of the lymph nodes in the head and neck.\nTo conserve periocular function and due to the advanced age of the patient, we planned micrographic surgery with secondary intention healing without primary suture rather than a wide excision (>2-cm margin), followed by further adjuvant radiotherapy. During the surgery, complete circumferential peripheral and deep free margins were confirmed by histological examination of frozen sections by a pathologist. Hematoxylin and eosin staining of the excised specimens showed a storiform malignant tumor, which was not connected to the epidermis and composed of tumor cells of variable size and appearance with hyperchromatic nuclei and abundant eosinophilic cytoplasm with frequent bizarre atypical cells and atypical mitoses (). The peripheral dermis of the tumor showed solar elastosis. Furthermore, focal neoplastic microinfiltrations were seen between bundles of skeletal muscle with moderate inflammatory cell infiltration (). Immunohistochemical staining demonstrated that the tumor cells were positive for vimentin () and CD68 (); weakly positive for smooth muscle actin (); and negative for pankeratin (), HMB-45, desmin, S-100 protein, c-kit, CD31, CD34, and CD99 (). On the basis of these pathologic findings, the diagnosis was revised to PDS. After four sessions of radiotherapy (total of 8 Gy in four fractions), the patient refused further radiotherapy due to neurological problems because of cerebral disease. The lesion healed completely without complications (), and there has been no evidence of local tumor recurrence during a 2-year follow-up despite no further therapy. |
A 32-year-old woman presented to our clinic with aesthetic concerns about her nose. The history of the patient revealed that she had no additional disease, no active drug use or allergy and no previous intervention to the facial area. On physical examination of the nose, the dorsal hump was inspected. Nasal speculum examination revealed that the bilateral airway was sufficiently open. The patient was prepared for rhinoplasty operation without additional radiological examination since the patient’s Cranial MR, which was recently taken for the headaches, showed no abnormalities for nasal airways and the patient had no problem with breathing. The preoperative photos taken during the patient’s initial application and MR images are shown in .\nThe patient was prepared for rhinoplasty operation under general anesthesia. Complete blood count, renal function tests, liver function tests, glucose and HbA1c levels, coagulation parameters and viral markers were within the normal range.\nOpen structural rhinoplasty was initiated with the elevation of the nasal flap with transcolumellar and infracartilaginous incisions. Cartilage excisions were performed in a way that the L shaped septum preserved with the safety margins of 1 cm caudally and cephalically. During the operation low to high lateral osteotomies were performed following the paramedian osteotomies and the nasal roof was closed. As a part of the operation, the inferior conchas were lateralized. The operation was terminated following the internal and external nasal splint applications. No abnormality was encountered intraoperatively.\nThe patient was discharged on the first postoperative day after appropriate medications were prescribed. The patient had no complaints during the controls performed on the 3rd, 5th and 8th postoperative days. The patient’s appearance on the 8th postoperative day is shown in and .\nWhen the patient was admitted to the postoperative 1st-month control, she had no complaints and it was observed that the postoperative edema and bruising were minimal. Post-operative 3rd and 12th-month routine controls were performed.\nThe patient was admitted to our clinic in the postoperative 15th month with complaints of deepening of the superior sulcus in the left upper eyelid and inward collapse in the left eye. On examination of the patient, no obvious pathology was detected in eye movements and visual acuity. The patient’s appearance in the 15th postoperative month is shown in and .\nInitially, the patient was consulted to the Ophthalmology department. Hertel exophthalmometry measurement was performed by the ophthalmologists. It was 17 mm on the right and 14 mm on the left side, and the significant difference was seen between two eyes. Other ocular examinations were normal, which included ocular eye movements, fundus examination, and visual acuity. Then, the patient was reevaluated using a paranasal CT. Radiological images in all cross-sections showed that the left frontal, the left ethmoid, and the left maxillary sinuses were opacified and the left orbital floor was located more inferiorly than the right one due to atelectasis. The images of the CT scan can be seen in .\nWith the current physical examinations and radiological imaging, the patient was diagnosed as Silent Sinus Syndrome and consulted with the ENT department. An antrostomy or antrectomy was recommended. |
A 25-year-old man was transferred to the emergency department by ambulance with an injury to the left ankle, after been involved in a road traffic accident (fall from own motorbike). He had sustained a minor injury to the head with an extensive blunt laceration of the soft tissues of the scalp (Glasgow Coma Scale, 15/15), a rib fracture accompanied by pneumothorax, as well as bruises and abrasions of the lower extremities. His primary complaint was severe pain and deformity of his left ankle. On physical examination, the ankle joint was noted to be deformed, swollen, and tender without lacerations. The skin temperature was normal, the sensory function could not be estimated because of pain, and there was motor loss. As the peripheral pulse was not palpable, the initial vascular status was assessed by applying the capillary nail refill test (observed). There is an obvious clinical deformity, and the ankle is revealed in a medial position with the talus head raised under the skin (). Radiographic evaluation of the ankle revealed an anteromedial dislocation of the talus, without any accompanying fracture (). Due to the absence of other fractures or neurovascular trauma, a closed reduction was attempted under general anesthesia with image intensifier fluoroscopy. Utilizing a technique previously described [], the talus was not able to be reduced to its anatomical position, neither was any sign of a “click” perceptible. A second attempt was also unsuccessful, and thus, a decision was made to perform an open reduction. Probably, the anteromedial dislocation of the talus, without malleolus fracture, was responsible for the unsuccessful closed reduction. The joint was explored through a dorsomedial incision (). The anatomical elements were identified, and the deltoid ligament and the tibiotalar syndesmosis were found to be intact. The displaced talus was easily reduced into the joint using gentle traction of the foot. The ankle and subtalar joints were stable (passive ankle dorsiflexion and plantar flexion), so Kirschner (K) wires were not used to hold the reduction. The anatomical reduction was confirmed by C-arm fluoroscopy (). The posterior tibial artery and dorsalis pedis artery pulse were felt following talus reduction. A posterior below-knee splint was applied with the ankle joint in the neutral position (). The patient remained hospitalized 15 days for observation, adequate trauma healing, and to prevent compartment syndrome. General measures for control possible infection, pain, edema, and decrease any late complications of the open reduction included antibiotic therapy (in the form of second-generation cephalosporins and amikacin intravenously), analgesics, non-steroidal anti-inflammatory drugs, elevation of the extremity, ice therapy, and ensuring the non-weight bearing status of the left foot. After 15 days of hospitalization, the patient was discharged home with a little skin necrosis over the wound and with oral antibiotic therapy for an additional 7-day therapeutic course, together with anticoagulant therapy for the prevention of embolic events. The patient remained non-weight bearing for 8 weeks, and the reduction was maintained in a posterior splint for 2 weeks from the time of the injury and a full plaster of Paris cast for another 6 weeks thereafter. Postoperatively, skin necrosis disappeared with the passage of time. At 9 weeks’ post-operative, he was allowed to partial weight bear with the use of a pair of crutches. At 11 weeks’ post-operative, the patient started a progressive rehabilitation program to facilitate a faster and safer recovery with better potential outcomes. The course of the post-operative rehabilitation program is summarized in . At the 6-week follow-up, a computed tomography (CT) scan with 3D reconstructed images of the ankle joint revealed the absence of joint deformity or osteochondral fractures. Small bone fragments were found in the posterolateral aspect of the ankle joint without affecting the motion of the joint (). At 12-week follow-up, a magnetic resonance imaging (MRI) scan showed that the bone bruises in the tibia, fibula, talus, and calcaneus had been resolved without apparent sequelae. At 3-month follow-up, the American Orthopedic Foot and Ankle Society (AOFAS) score was 49/100. At 6-month follow-up, the patient was almost asymptomatic and had a good range of motion at the ankle and subtalar joint. He wore regular shoes and had resumed his regular daily activities. Radiographs showed no signs of avascular necrosis of the talus. At 6-month post-operative follow-up, a significant improvement of AOFAS score from 49 points to 88 was observed. After 6 months, the patient was lost from the follow-up and maybe he returned to his home place far away from our clinic when his general condition allowed it.operative, he was allowed to partial weight bear with the use of a pair of crutches. At 11 weeks’ post-operative, the patient started a progressive rehabilitation program to facilitate a faster and safer recovery with better potential outcomes. The course of the post-operative rehabilitation program is summarized in . At the 6-week follow-up, a computed tomography (CT) scan with 3D reconstructed images of the ankle joint revealed the absence of joint deformity or osteochondral fractures. Small bone fragments were found in the posterolateral aspect of the ankle joint without affecting the motion of the joint (). At 12-week follow-up, a magnetic resonance imaging (MRI) scan showed that the bone bruises in the tibia, fibula, talus, and calcaneus had been resolved without apparent sequelae. At 3-month follow-up, the American Orthopedic Foot and Ankle Society (AOFAS) score was 49/100. At 6-month follow-up, the patient was almost asymptomatic and had a good range of motion at the ankle and subtalar joint. He wore regular shoes and had resumed his regular daily activities. Radiographs showed no signs of avascular necrosis of the talus. At 6-month post-operative follow-up, a significant improvement of AOFAS score from 49 points to 88 was observed. After 6 months, the patient was lost from the follow-up and maybe he returned to his home place far away from our clinic when his general condition allowed it. |
A 55-year-old incarcerated male presented to the emergency room with a two-week history of left-sided scrotal pain and swelling.\nThis patient had a history of prostate cancer and high-grade urothelial bladder cancer. His prostate cancer was diagnosed 15 years prior to the current presentation, managed with radiation, and had since remained stable. The patient's high-grade, high-risk bladder cancer was diagnosed two years prior to current presentation. At the time, the patient initially presented with hematuria. A CT urogram performed at the time revealed a nonspecific bladder mass. Biopsy of the mass confirmed urothelial carcinoma. The patient's therapy course included a transurethral resection of the bladder tumor (TURBT), intravesical mitomycin, interferon alfa-2b, and intravesical BCG therapy. He received a total of seven intravesical injections of 50 mg live BCG per injection (350 mg cumulative dose) over the course of 18 months.\nThe patient's other significant medical history included rheumatoid arthritis, for which the patient was on weekly methotrexate and daily tofacitinib treatment. Both medications were discontinued on admission.\nThe patient developed scrotal pain two weeks prior to admission. He initially presented to the emergency department with this pain. At that time, he was diagnosed with acute bacterial epididymitis and was prescribed a course of ciprofloxacin. He had no improvement in his symptoms despite this treatment. Over the subsequent days, he reported experiencing intermittent chills and night sweats. He denied any penile discharge or any history of a sexually transmitted disease.\nEvaluation of the testicle with ultrasound revealed multiple diffuse nodular areas of hypoechogenicity (Figure , ), as well as marked hypervascularity involving all of the left-sided structures (Figure , ). Routine blood and urine cultures were negative, and mycobacterial blood and urine cultures, as well as bladder biopsy, were pending as of this writing.\nAntituberculous therapy was started based on the following considerations regarding the patient's presentation. The lack of observed response to ciprofloxacin therapy decreased the likelihood of uncomplicated bacterial orchitis. We considered this patient's history of bladder cancer involving multiple BCG treatments and the increased likelihood of BCG orchitis. Possibly, his immunocompromised state secondary to the malignancy, in combination with mitomycin chemotherapy and immunomodulatory rheumatoid arthritis treatment, could have increased his susceptibility to a mycobacterial infection. Finally, as ultrasound findings were characteristic of BCG orchitis, the patient was promptly initiated on a combination therapy with levofloxacin, rifampin, isoniazid, and ethambutol. The patient showed marked improvement in pain control and testicular swelling over the following weeks. |
A 30 year-old man was admitted to the hospital Emergency Department with high fever (37.5°C-40°C), vomiting, slurred speech, and mild cognitive impairment. He had been in Spain and the UK on business from February 23rd to March 1st, 2020 and experienced high fever and vomiting 3 days after returning to Japan. He was treated at a hospital on the fifth day after the symptom onset, but his symptoms persisted, and he was unable to retain any solids or liquids because of vomiting. His parents living in a distant part of the country noticed his slurred speech and called the emergency services on the seventh day after the onset of symptoms.\nThe patient was slightly confused on admission and showed mild cognitive impairment with slurred speech. He also had a brief convulsion episode on the first hospital day. Physical examination did not show any abnormalities of muscle tone, tendon reflex, and any pathological reflexes. We assessed his consciousness level using GCS14 or JCS I-1. His other vital signs, CBC, biochemical data, and arterial blood gas analysis are presented in .\nA chest CT showed multiple areas of bilateral peripheral ground glass infiltration of the lungs (). A brain MRI revealed no focal signs suggesting stroke and no encephalopathic signs such as encephalitis or meningitis. Taken together, we suspected COVID-19 and performed PCR for SARS-CoV-2 using a nasopharyngeal swab and a CSF sample. The swab tested positive, but the CSF was negative. The CSF data were as follows: clear appearance, 12.5 cmH2O initial pressure, nuclear cell count <1.0, 63 mg/dL glucose, and 43 mg/dL protein ().\nFrom these data, we hypothesized that his neurological abnormalities were not directly caused by infection of the CNS by SARS-CoV-2 but by metabolic abnormalities such as a low serum sodium level. Accordingly, we corrected the sodium level by saline infusion, and by the fourth hospital day, his consciousness was almost completely normalized as his body temperature decreased and sodium level increased (141 mEq/L Na) (). On the 13th hospital day, he was discharged after twice confirming SARS-CoV-2 negativity by PCR.\nWritten consent, including that for radiographic data, was obtained for publication. |
A 19-year-old Japanese woman presented to our hospital complaining of a mass in the middle of the pharynx, but without any systemic signs or symptoms. A mass measuring approximately 25 mm in diameter was noted in the middle of the pharynx. The mass was elastic in consistency, tender, and mobile, with a normal overlying oral mucosa. The patient's general condition and family history were unremarkable. A plain computed tomographic (CT) scan of the neck showed a low-density, homogeneous, neoplastic lesion occupying the right parapharyngeal space. A slight partial contrast effect was observed. Magnetic resonance imaging (MRI) showed the tumor to have low signal intensity on T1-weighted images and heterogeneously high signal intensity on T2-weighted images. The tumor exhibited an uneven contrast effect on the T1-weighted coronal images (Figure ). A fine-needle aspiration biopsy was performed to rule out any malignancies but was inadequate to make a definitive diagnosis. For definitive diagnosis and treatment, a total resection of the tumor was performed while the patient was under general anesthesia. Complete excision using a transoral approach was possible by the use of a Weerda laryngoscope. Specifically, as in an excision of the palatine tonsils, we used a Davis mouth gag to obtain a clear field of view of the oropharyngeal area and then made a longitudinal incision in the center of the tumor to separate it from the mucosa (Figure ). However, the surgical field became difficult to visualize adequately while the deep portions and the outside of the tumor were separated, so the field of view was expanded by inserting a Weerda laryngoscope and slightly opening its tip, thereby making a total excision possible (Figures and ).\nA gross pathological examination revealed a well-circumscribed 72 × 33 × 11-mm tumor with a fibrous capsule. The cut surface was whitish and exhibited a whorled pattern. A histopathological examination showed the tumor to be composed predominantly of nodular neurophilic ganglioneuromatous stroma with a minor component that consisted of collections of maturing ganglion cells which were unevenly distributed. The fibrous capsule of the tumor was thinned in areas, but no extracapsular extension was identified. The histological findings were consistent with ganglioneuroma, maturing subtype (Schwannian stroma-dominant neuroblastic tumor) (Figure ).\nAfter surgery, the patient exhibited left palpebral ptosis and anisocoria with ipsilateral mydriasis typical of Horner's syndrome. These symptoms resolved completely within three months.\nUltrasonography of the abdomen was performed to exclude any visceral involvement. The patient's postoperative course was satisfactory, and she was discharged with no difficulties. No local recurrence or distant metastases have been observed during the five years since her surgery. |
A 50-year-old post-menopausal woman was diagnosed with a stage IIB (T2N1M0) right breast cancer and subsequently years later developed an unusual recurrence that was diagnosed following rupture of one of her breast prostheses. Following initial diagnosis, she underwent a right modified radical mastectomy and review of surgical pathology revealed a grade 4 (of 4) invasive lobular carcinoma, nuclear grade 2 (of 3), forming a 2.2 × 2 × 1.8 cm mass. No definite vascular invasion was noted apart from the central tumor mass, although lobular carcinoma in-situ with extension into adjacent ducts was seen. Lactiferous ducts beneath the nipple showed pagetoid spread of carcinoma cells. One of 14 right axillary lymph nodes was positive for metastatic involvement with focal extranodal extension of disease. Tumor cells were ER/PR positive. Following surgery, she received six months of chemotherapy with cyclophosphamide, methotrexate and 5-fluorouracil (CMF). A subsequent prophylactic left simple mastectomy with bilateral breast reconstruction was performed 4 months following completion of chemotherapy. Approximately 4 months after surgery, tamoxifen therapy was started and administered for 5 years. Of note, this patient underwent a total abdominal hysterectomy and bilateral salpingo-oophorectomy a year after breast reconstructive surgery.\nThe patient had regular follow up without evidence of disease recurrence. Approximately 12 years after her breast reconstructive surgery, she developed a deflated right breast implant. She was scheduled for bilateral implant exchange surgery. During preoperative evaluation, she was found to have evidence of mitral valve regurgitation due to a flail mitral valve posterior leaflet, and subsequently underwent mitral valve repair. The cardiothoracic surgeon informed the patient that her sternum was found to be “somewhat mushy” during her sternotomy.\nAbout 5 months after open heart surgery, the patient had developed a neck lump and back pain. Imaging studies with CT revealed postoperative mastectomies with implants. However, the right breast implant was ruptured with extensive soft tissue mass and nodularity involving the anterior chest wall, predominantly anterior to both sides of the sternum but slightly more marked on the right with subcutaneous nodularity throughout the right mastectomy site (). This was noted to be inseparable from the adjacent pectoralis muscles along with right subpectoral adenopathy and right neck base adenopathy consistent with tumor recurrence. The anterior chest wall mass extended posteriorly through the chest wall into the hemithorax and was also associated with internal mammary adenopathy. Partial lytic lesions were seen in the mid sternum. In addition, there was bulky anterior mediastinal adenopathy and tumor extending inferiorly along the anterior pericardium and anterior to the right atrium and right ventricle as well as to the root of the aorta (). Nodularity was noted in the right upper lung pleura and left lung base pleura. There were bulky soft tissue masses in both costophrenic angles. Tumor nodularity was noted anterior to the liver representing peritoneal implants. Skin thickening was noted over both anterior chest walls but greater on the right. Bony metastases were noted in the T5 and L1 vertebral bodies, the right temporal bone of the skull, and the right anterior iliac bone.\nThe patient underwent a T10 vertebroplasty and then subsequent palliative radiotherapy to T8 through L1 vertebral bodies. During palliative radiotherapy, she developed right hip pain and was found to have a destructive metastasis in the right femoral head and neck requiring surgery with a right hip replacement followed by palliative radiotherapy to bilateral hips and the right femur. She went on to receive palliative chemotherapy but ultimately expired from disease progression approximately 11 months following diagnosis of metastatic disease. |
A 36-year-old woman at 9 weeks' gestation was transferred to our facility with severe posterior neck pain. She presented with a four month history of severe pain from the back of both ears to the ridge of the shoulder with symptoms gradually worsening at eight weeks of pregnancy. She also complained of numbness in fingers of both hands and difficulty in raising the right upper extremity. No significant muscle weakness in the lower extremities was identified. No unusual findings on cervical radiographs and computed tomography (CT) images were found. Magnetic resonance imaging (MRI) revealed an oval-shaped tumor (size: 30 × 15 mm) in the intradural space with a T1 – weighted image (WI) low and a T2 – WI high at the level from the C2 to C3 vertebrae. MRI showed that the spinal tumor severely compressed the spinal cord (Fig. ). The initial diagnosis was an intradural extramedullary tumor.\nBecause of the patient's severe pain and progressing neurological symptoms, the patient felt that it was difficult to wait for the surgery until after delivery. The therapeutic strategy was determined by consulting with obstetric and anesthesia teams. Based on the results of consultations, the spinal cord tumor resection under general anesthesia at 12 weeks of gestation was planned. The patient and her family received sufficient explanation for the necessity of surgery, the method of general anesthesia, possible risks of the surgery, and complications. With an adequate understanding of the surgical treatment, the patient provided informed consent. Under general anesthesia with the use of intraoperative neurophysiological monitoring, a hemi-laminoplasty of C2 to C3 was performed in a prone position, and the spinal tumor was resected totally under microscopy (Fig. ). Inter-lamina spacers (Centerpiece®, Medtronics, City, State, USA) were placed at the C2 and C3 lamina, followed by a dura suture (Fig. ). Motor-evoked potentials (MEPs) were monitored continuously during surgery; no significant changes in MEP amplitude were observed.\nHistology (Hematoxylin & Eosin staining) of the removed tumor showed spindle-shaped cells in multiple directions in a myxoid collagenous background (Fig. a). The pathological diagnosis following surgery was schwannoma. The patient's neurological symptoms were significantly improved post-surgery. The post-operative MRI showed no residual tumor and the spinal cord was released (Fig. ). The post-operative course was uneventful and the patient was discharged on post-operative day 14. Six months following the operation, she delivered a healthy baby in her 40th week of pregnancy. At a 12-month follow-up, the patient had no neck pain or neurological findings and no abnormalities were observed during medical examinations of both mother and child.\nImmunohistochemistry was performed on formalin-fixed, paraffin-embedded tissues, as previously reported. In short, following endogenous peroxidase inactivation and heat-induced epitope retrieval, the sections were stained with estrogen receptor α antibody (M7047: Agilent, Santa Clara, CA, USA), estrogen receptor β (M7292: Agilent) and progesterone receptor (MA5-14505: Invitrogen, Carlsbad, California, USA). Mouse IgG (Agilent) was used as the isotype or negative control. The sections were visualized using the universal immuno-enzyme polymer method (Histofine Simple Stain MAX-PO; Nichirei Biosciences, Tokyo, Japan) and 3,3′-diaminobenzidine tetrahydrochloride (DAB; Dojindo, Tokyo, Japan), followed by counterstaining with Mayer's hematoxylin. Weak immunoreactivity against the estrogen α receptor was found on the nuclei and in the cytoplasm of schwannoma cells (Fig. b). On the other hand, intense immunoreactivity against the estrogen β receptor was clearly identified on the nuclei and in the cytoplasm of schwannoma cells (Fig. c). No significant immunoreactivity against the progesterone receptor was identified (Fig. d). No immunoreactivity was found in the isotype (negative) control (Fig. e). |
A 49-year-old man with a background of hypertension and hypercholesterolaemia presented with dyspnoea and exertional chest pain. Coronary angiography () demonstrated an RCA aneurysm in a right-dominant coronary system. Computerised tomographic (CT) coronary angiography () and three-dimensional reconstruction of CT images () further characterised the aneurysmal RCA to be arising from the normal position on the aorta with fistulous connection to the RA. Transesophageal echocardiography demonstrated preserved biventricular function with a mildly dilated right ventricle (RV) and no significant valvular abnormalities. The coronary sinus was dilated to 40 mm but agitated saline (bubble) test was negative for persistent left superior vena cava. Following multidisciplinary discussion, informed consent was obtained for surgical intervention on symptomatic and prognostic grounds.\nAt operation, the aneurysmal RCA was exposed following median sternotomy (). It was dilated at 4 cm and tortuous with a normal course along the anterior atrioventricular groove descending down towards the crux but extending further and then turning back to drain into the RA just anterior to the insertion of the inferior vena cava. The posterior descending artery (PDA) arose from the distal aspect of the artery. Cardiopulmonary bypass was established with standard ascending aortic and bicaval cannulation, with snares around the venous cannulae. Initially, 1.5 litres of antegrade cold-blood cardioplegia was delivered into the aortic root, followed by intermittent infusion at 20-minute intervals. Following right atriotomy, two fistulous connections identified between the aneurysmal RCA and the RA were oversewn with a 4-0 polypropylene suture followed by closure of the right atriotomy. A bypass graft from the ascending aorta to the PDA was performed with a segment of long saphenous vein. The RCA was flush ligated at its ostium and multiple ligations were performed downstream with heavy silk ties (). The patient was weaned off cardiopulmonary bypass with stable haemodynamics without any inotropic support.\nOn skin closure, the patient developed unexpected ventricular fibrillation (VF). External direct-current cardioversion (DCCV) was successfully performed, although the patient then developed recurrent episodes of VF. A lignocaine infusion was commenced alongside emergent resternotomy and internal DCCV with eventual restoration of sinus rhythm. Satisfactory blood flow was observed in the saphenous vein graft, although right ventricular and septal hypokinesia were demonstrated on transesophageal echocardiography, consistent with right ventricular stunning. The right pleura was opened and the sternum stented open to relieve any potential cardiac compression, and milrinone and noradrenaline infusions were commenced. The patient was transferred to intensive care with stable haemodynamics. Following uncomplicated sternal closure, the patient was extubated on the following day. He made an uneventful recovery and was discharged home seven days after surgery. |
A 19-year-old male of Caucasian origin was admitted to our center as a polytrauma after a road traffic accident. He was previously fit and well, a nonsmoker with an alcohol intake of approximately 10 units per month. The accident, in which his motorcycle collided with an oncoming vehicle, caused him to sustain multiple significant injuries including unstable pelvic fractures and femoral fractures. He had bilateral pneumothoraces, extensive pulmonary contusion, and a splenic hemorrhage. He presented in extremis with signs of hypovolemic shock. He was intubated and resuscitated using local major hemorrhage protocols to achieve a blood pressure of 159/93, receiving ten units of packed red cells and four units of fresh frozen plasma in the emergency department.\nHe underwent an emergency laparotomy and splenectomy and was subsequently transferred to the intensive care unit, where he became increasingly hypoxic with features of adult respiratory distress syndrome (ARDS). This culminated in him receiving veno-venous extracorporeal membrane oxygenation (ECMO) from day 15 of his admission for 21 days. He returned to theater on day 21 for a massive haemothorax which required an emergency thoracotomy. After being decannulated from the ECMO circuit, he was stepped down to the general intensive care unit on day 36 and was transferred to the ward on day 55 before being discharged after a 4-month admission including a prolonged rehabilitation and recovery period.\nAfter presentation and commencement of ECMO, there was a relatively modest change in liver function tests. Alkaline phosphatase (ALP) increased from 55 to 143 IU/L between day 1 and day 6 of hospital admission and no persistent alanine transaminase (ALT) rise until after decannulation. Proceeding decannulation, there was a sequential increase in ALP peaking at 2335 IU/L on day 113. ALT rose to a lesser extent, peaking at 781 IU/L on day 52. The bilirubin did not rise above 57 μmol/L. The pattern of liver function tests is summarized in Figure . Autoantibody screen, immunoglobulins, and viral hepatitis serology were negative.\nSerial ultrasound, computerized tomography (CT), and magnetic resonance imaging excluded biliary stones and sludge. The liver on CT at day 1 showed normal liver and biliary structure (Figure ), and it was not until 10 months after the admission, the repeat magnetic resonance cholangiopancreatography (MRCP) demonstrated a multistenotic pattern of disease within the intrahepatic ducts (Figure ).\nIn the absence of significant casts within the biliary tree and no evidence to suggest biliary sepsis, endoscopic retrograde cholangiopancreatography (ERCP) was felt not to be helpful. A conservative management approach was taken, and the patient was instigated on ursodeoxycholic acid to help improve cholestasis. With bilirubin improving, the patient was closely monitored as an outpatient upon discharge for progressive liver disease and dysfunction. Repeat imaging and noninvasive fibrosis assessments were undertaken. Despite having evidence of SSC, the synthetic liver function has remained excellent, cholestasis markers have improved, and there have been no episodes of cholangitis or biliary sepsis for over 1 year. |
A 31-year-old man (180 cm, 87 kg) was admitted to our hospital for the extraction of horizontally impacted wisdom teeth in the bilateral mandible. His past history included no appreciable disease and no evidence of neck or head injuries. The preoperative neurological and anesthetic evaluation results were all within normal limits (American Society of Anesthesiologists physical status 1 and Mallampati score grade 1). Tooth extraction was conducted under general anesthesia. Anesthesia was induced by the intravenous administration of propofol and remifentanil, with neuromuscular blockade obtained with rocuronium. Transnasal intubation using a Macintosh laryngoscope (blade size 3, ACOMA Medical, Tokyo, Japan) was performed gently during the first attempt without any difficulty by a dental anesthesiologist with 6 years of experience in the field. A nasotracheal tube (Portex® Ivory PVC Soft Seal cuffed North Polar endotracheal tube; Smiths Medical Japan, Tokyo, Japan) with an internal diameter of 7.0 mm and an external diameter of 10.2 mm were secured at a depth of 29 cm in the left nasal cavity. No problem was encountered during intubation (Cormack grade 1). The cuff was inflated to a pressure of approximately 20 cm H2O, and the cuff pressure was continuously monitored and adjusted between 20 and 25 cm H2O during the surgery. General anesthesia was maintained with propofol and remifentanil. Mechanical ventilation was set as follows: a tidal volume of 800 ml and a respiratory rate of 10/min. With the use of a tongue depressor, a throat pack was inserted on the posterior part of the tongue to avoid the passage of blood into the respiratory tract. The surgery was carried out with the patient in a supine position with the neck slightly extended. During the surgery, the head and neck positions were changed from side to side several times, and no significant changes in respiratory and cardiovascular parameters or neurosurgical abnormalities occurred. At the end of the surgery, extubation of the nasotracheal tube was performed after the removal of the throat pack without any problems. Neuromuscular blockade was not reversed. The durations of the surgery and anesthesia were 221 min and 275 min, respectively. After a short observation in the operating room, the patient was transported to the ward.\nAfter returning to the ward, the patient experienced hoarseness and difficulty swallowing. X-ray and computed tomography (CT) revealed an alveolar infiltrative shadow. In addition, an endoscopic evaluation showed unilateral vocal cord paralysis and aspiration, leading to a diagnosis of aspiration pneumonia. Treatment with intravenous sulbactam/ampicillin (SBT/ABPC) (6 g/day) was initiated immediately. Furthermore, since the tongue deviated towards the right upon protrusion, multiple cranial neuropathy was suspected, which led to a request for detailed otorhinolaryngological and neurological examinations. The results of the examinations led to a diagnosis of bilateral glossopharyngeal and vagus nerve paralysis, right recurrent nerve paralysis, and right hypoglossal nerve paralysis. The patient was not able to swallow at all. The patient underwent nonsurgical management with vitamin B12 (1500 mcg/day), together with speech and swallowing therapy. A nasogastric tube was placed to prevent aspiration and to supplement oral intake. The results of a meticulous neurologic examination of the other cranial nerves, including CT and magnetic resonance imaging (MRI), showed no evidence of the involvement of the central nervous system. Aspiration pneumonia gradually improved, with cycles of improvement and worsening. All cases of nerve paralysis improved in approximately 6 months, and no complications remained. |
Our patient is a 67-year-old male with a history of hypertension, type 2 diabetes, and hyperlipidemia, who went through the Inova Movement Disorders Program due to a history of fluctuating, progressing uncontrollable movements of the left side of his body. He reports these movements had been present for around 10 years, worsening over time.\nAt baseline, he always felt that his left arm and leg were moving, at times exhibiting large amplitude movements which could knock things down or affect his walking, making him fall. He described it as abnormal, uncontrollable movements that made his arm ‘reach around or fling out’ His leg was also described to ‘dance on its own.’ While at baseline, these were present but minimal stress or anxiety would exacerbate the movements. There were no movement issues on his right side, nor any facial movements noted.\nHis exam showed episodic, mild with some interspersed moderate-amplitude quick movements of the left hand, forearm, proximal arm muscles, as well as movement of the left leg. These were not suppressible, brought out more by distraction or with anxiety, such as discussing an MRI scan as he was claustrophobic. He also demonstrated some lateralizing movements of the head to the left, but no facial or tongue movements. His right side showed no such signs, and his neurological exam was otherwise unremarkable.\nHe was sent for a CT scan of the head initially due to a fear of MRI scanners, and was found to have asymmetric calcification of the right caudate nucleus and anterior putamen (Figure ). The calcification spared the anterior limb of the internal capsule. Laboratory work for parenchymal calcification was then sent and was normal. The MRI of the brain with contrast demonstrated both hypointense gradient echo (GRE) signal in the right caudate nucleus and putamen, as well as an underlying DVA (Figures -).\nGiven the lateralizing of his deficits and correlation with opposite hemispheric parenchymal calcification, he was diagnosed with unilateral hyperkinetic movements as a product of putamen and caudate calcification from an underlying DVA. Consideration was made for focal hemorrhage, but presentation and imaging were not thought to reflect this pathology. He was then treated with 0.25 mg clonazepam 2x daily with significant improvement in his hyperkinetic movements. |
A 43-year-old man presented to the outpatient clinic with complaints of swelling in the oral cavity and discomfort during swallowing for the past 1 month. He complained of pain only when the lesion was poked. There was no history of paraesthesia. Medical history was noncontributory. Inspection of the oral cavity revealed a solitary sessile swelling of size 2 × 2 cm and oval in shape in the region of the right soft palate extending into the hard palate. Surface of the mucosa over the swelling was smooth, with mild erythema on the superior surface. The margins were well defined with no evidence of any secondary changes. On palpation, it was nontender and firm in consistency []. The regional lymph nodes were not palpable. A provisional diagnosis of benign salivary gland neoplasm probably adenoma was made. The probable differential diagnoses considered were fibroma, neurofibroma, retention cyst and infected palatal cyst. Further investigations were done. Intraoral radiograph in the region of the left upper posterior teeth did not reveal any significant pathology []. Occlusal radiograph [] and orthopantomograph [] showed the presence of a periapical radiolucent lesion in relation to nonvital left upper central incisor. No features of erosion of the palatal cortex were noticed in the occlusal radiograph and orthopantomograph. Contrast computed tomography (CT) revealed enhanced soft tissue density with attenuation of +30 Hounsfield units in the left posterior palate region suggestive of a soft tissue mass probably of muscular or neuronal origin []. Incisional biopsy was done, and the tissue was sent for histopathological examination. Histopathological examination revealed the presence of characteristic Antoni A and Antoni B areas blending imperceptibly [Figures and ]. Antoni A areas were composed of compact spindle cells with wavy nuclei and indistinct cytoplasmic borders. They were arranged in short bundles or interlacing fascicles. Highly differentiated Antoni A areas with nuclear palisading, whorling of cells and Verocay bodies were also observed, Verocay bodies are two compact rows of cells with well-aligned nuclei separated by homogeneous eosinophilic areas. Antoni B areas were less orderly and less cellular. The spindle or oval cells were arranged haphazardly in the loosely textured matrix composed of delicate collagen fibers. Mild, diffuse inflammatory cell infiltrate is seen. An immunohistochemical examination of the tumor showed strong and uniform positivity with S100 antigen []. Clinical behavior, histopathological findings and immunohistochemistry confirmed the diagnosis of schwannoma.\nThe lesion was removed under general anesthesia []. Gross inspection of the lesion revealed a grayish, well-circumscribed mass, approximately 3 cm × 2 cm × 2 cm in size []. Histopathology of the excised specimen also confirmed as schwannoma. After 6 months of follow-up, no evidence of recurrence was detected. |
A 70-year-old man was admitted to our hospital for the evaluation of a pancreatic mass that was incidentally detected during a screening abdominal sonographic examination. He was asymptomatic and he did not have any specific medical history. He had a habit of drinking one or two cups of alcohol daily for several decades. The laboratory tests at the time of admission revealed that the CA19-9, CEA, alpha-fetoprotein, amylase and lipase levels were within the normal limits. The ultrasonographic images showed a hypoechoic mass in the uncinate process of the pancreas, and this was not accompanied by dilatation of the main pancreatic duct. There was no discernible vascularity within the mass on the color Doppler image (). We then performed contrast-enhanced multidetector-row CT. The contrast-enhanced dynamic CT images revealed two round masses with one each in the uncinate process (about 2 × 1.8 cm for the mass size) and tail (about 1.8 × 1.5 cm for the mass size) of the pancreas; these isodense masses on the unenhanced CT images were slightly hypodense, slightly hyperdense and slightly hyperdense, respectively as compared to the pancreas parenchyma on the contrast-enhanced arterial, portal, and delayed-phase CT images that were obtained at 30 seconds, 75 seconds and 180 seconds, respectively, after the injection of contrast agent (). There were no enlarged lymph nodes in the abdomen. MR imaging was performed to further evaluate these pancreatic masses. The MR images demonstrated that the two masses were hypointense and hyperintense on the T1-and T2-weighted images, respectively (). Although subtle enhancement of the two masses was detected, these lesions were shown to be of slight hypointensity as compared to the intensity of the pancreas parenchyma on the gadolinium-enhanced arterial phase T1-weighted image obtained at 25 seconds after the injection of contrast agent (). Meanwhile, the mass lesions were more enhanced than the pancreas parenchyma on the portal and delayed-phase T1-weighted MR images () obtained at 60 seconds and 180 seconds, respectively, after the injection of contrast agent.\nSeveral imaging findings of these tumors, such as the delayed enhancement, the double lesions and the normal caliber of the main pancreatic duct, resulted in the preoperative tentative diagnosis of neuroendocrine tumor or lymphoma. Under this impression, endoscopic US-guided biopsy was performed, but this failed. Finally, total pancreaticoduodenectomy was performed for these masses because the malignant potential of these masses could not be ruled out.\nThe gross surgical specimen showed two well circumscribed yellowish masses in the uncinate process and tail of the pancreas (). Microscopic examination revealed lymphoid follicles with enlarged germinal centers and dense, rich collagenous fibrous tissue admixed with a lymphoplasma cell infiltration (). The immunohistochemical stains demonstrated that the follicular lymphocytic infiltrate of a polyclonal origin was composed of germinal centers with cells expressing B-cell characteristics and these germinal centers were surrounded by rims of cells expressing T-cell characteristics. No immunoreactivity for IgG4 was noted. These histologic features resulted in the diagnosis of localized lymphoid hyperplasia of the pancreas. He had an uneventful postoperative course and he has been alive for 18 months after surgery. |
A 50-year-old male physician, with no medical comorbidities, presented to the emergency room with history of forceful unrelenting vomiting associated with moderate nausea that had progressed over the past 2 weeks. Initially attributed to gastritis, his symptoms did not improve with proton pump inhibitor and antiemetic medications.\nHis level of consciousness had started to drop over the preceding 24 hours with significant increase in the severity of his headache. Physical exam showed a depressed Glasgow coma score (GCS) of 12 equally reactive pupils with no focal neurological deficit.\nAn urgent brain computed tomography (CT) scan revealed obstructive hydrocephalus with an infratentorial somewhat hyperdense lesion in the fourth ventricular that enhanced homogeneously ().\nThe patient was admitted and urgent endoscopic third ventriculostomy was performed, with rapid recovery of his level of consciousness postoperatively. Cerebrospinal fluid (CSF) analysis and cytology results were within normal and negative for malignant cells.\nFurther brain and spine imaging with magnetic resonance (MR) scan demonstrated a 20 × 25 × 30 mm strongly enhancing mass occupying the inferior half of the fourth ventricle (Figures and ). The mass acquired the shape of the ventricular cavity, extended towards the lateral foramina of Luschka and inferiorly towards the obex without evidence of fourth ventricular dilation. No evidence of parenchymal invasion or drop metastases was seen on imaging of the whole spine. The radiologist's impression was that of an ependymoma or possibly a subependymoma.\nThe patient was then shifted, 2 days later, to the operating theatre for resection of a tumor of the fourth ventricle. Midline posterior fossa craniotomy was performed and the tumor was accessed through a transvermian approach. The tumor was exophytic, grayish in color, firm, and fairly vascular, being fed by leptomeningeal vessels arising from top of the brainstem. Invasion of the lower pons and upper medulla oblongata in addition to the superior vermis was noted intraoperatively.\nInitially, biopsy was taken and frozen section was suggestive of malignant neoplasm with differential diagnosis including medulloblastoma and ependymoma. Complete resection was proceeded using ultrasonic aspiration and was attainable utilizing an easily identifiable tumor-brainstem interface.\nHistopathology would show the cells positive for CD45 (Leukocyte Common Antigen) as well as for CD79a, CD20 (B-cell antigens), and HLA-DR and focally positive for CD10 and vimentin. The morphology and immunohistochemical profile was consistent with a high-grade B-cell lymphoma (Figures and ).\nConsequent to receiving the histopathology results, staging workup consisting of neck, chest, abdomen, and pelvis CT studies would show no disease elsewhere and HIV tests were negative.\nThe patient has a smooth postoperative period and his vomiting and gait imbalance had markedly improved.\nHe received a 6 cycles of intravenous and intrathecal methotrexate with subsequent whole brain radiotherapy and boost to the surgical bed of total of 30 Gys. Both immediate and 18-month postoperative brain and whole spine MR imaging scans showed no recurrence (Figures and ). |
An 18-years-old girl diagnosed at the age of three years to have right otitis media with effusion (OME) was treated medically. She presented again at the age of 8 years with the same complaint but did not respond to the medical treatment and underwent right myringotomy with ventilation tube and adenoidectomy. Two years later, her symptoms recurred and underwent right T-tube insertion. Symptoms had recurred again after removal of the T-tube 2 years later.\nShe presented at the age of 18 years with right hearing loss and intermittent nonpulsatile tinnitus. There was no history of otorrhoea, otalgia, or vertigo, and there were no abnormal neurological signs or symptoms.\nPhysical examination revealed retracted right tympanic membrane, while the left was normal. Audiogram showed right moderate conductive hearing loss (CHL) () with shallow type-A tympanogram.\nComputed tomography (CT) of the temporal bone () showed right mastoid and middle ear mass with intracranial extradural extension elevating the right temporal lobe of the brain. The lesion was associated with marked erosion of the floor of middle cranial fossa.\nMagnetic resonance imaging (MRI) of the temporal bone and brain with and without gadolinium () revealed T1 and T2 sequences of heterogeneous but predominantly hyperintense mass located mainly in the right mastoid and middle ear cavity causing elevation of the right temporal lobe of the brain. Initial diagnosis of congenital cholesteatoma with erosion of floor of middle cranial fossa and extracranial extension was made.\nThe patient underwent a combined approach: right temporal craniotomy with middle cranial fossa approach to excise this mass by the neurosurgeon, associated with canal wall down mastoidectomy by the otologist, which was necessary to eradicate the whole mass. The mass was encapsulated and adherent to the dura of the middle cranial fossa. Incision of the mass produced yellowish cheesy content with hair. A whitish mass was occupying the antrum and mastoid air cells. No incus or stapes suprastructure was seen, and the proper middle ear cavity was intact. The patient tolerated the procedure well and recovered with no complications.\nHistopathological report () showed dermoid cyst. Three months later, the CT with and without contrast and MRI with and without gadolinium of the temporal bone and brain () revealed complete resection of the dermoid cyst and return of the right temporal lobe of the brain to its normal position. |
A 67-year-old Caucasian man presented to the emergency room because of a 4 days’ history of abdominal pain, with one episode of vomiting.\nThe patient’s past medical history was significant for colonic diverticulosis and an episode of gastrointestinal bleeding one year before. The event had been investigated by two different gastroscopies, a colonoscopy and a MDCT, which produced inconclusive results. Ten months later he was newly admitted because of abdominal pain and fever at 38.0 C°, with valid urination and defecation. A CT of the abdomen was performed, which confirmed the colonic diverticulosis and revealed the presence of multiple diverticula of the small intestine, fat stranding, signs of inflammation as well as a small amount of free liquid in the abdomen. The patient was hence diagnosed with jejunal diverticulitis and managed conservatively with intra-venous antibiotics, with an apparent complete recovery.\nHe re-presented to the emergency department two months later with acute abdominal pain. The pain was described as severe and constant, localized mainly in the lower abdomen with clinical signs of peritonitis. No change in bowel habits nor urinary symptoms were complained. His vital signs were stable, with a temperature of 37.2 C°; he appeared fully oriented and not in any acute distress. Laboratory examination reported a hemoglobin of 121 g/dL, a WBC count of 12.2 × 10E9/L and a CRP of 249 mg/L. Other laboratory data were within normal limit. An abdominal and pelvic contrast-enhanced computed tomography, with administration of oral contrast, was performed. Jejunum and ileum showed several diverticula as well as an inflammatory thick-walled mass involving different loops of the intestine. In addition, free fluid in the abdomen and a small amount of subdiaphragmatic air were reported (, ). On the basis of these findings, the diagnosis of perforated diverticulitis was hereby proposed.\nThe patient underwent a diagnostic laparoscopy which revealed plenty of purulent yellowish liquid collected in the right abdomen and a conglomerate of intestinal inflamed loops. We hence decided to convert immediately the procedure to laparotomy. Large multiple diverticula were found covering a section of small intestine approximately 2.5 m long, without signs of obvious macro perforation. Among the middle distal tract of the jejunum and the middle distal tract of ileum, strong adhesions were identified (). The involved segments of jejunum and ileum were connected by an intestinal loop free of signs of diverticulosis (). There were no signs of bowel ischemia. Adhesiolisis was partially carried out, however, because of difficulties associated with the procedure, we opt to perform a double enterectomy, removing only those segments involved in the intestinal conglomerate and deeply affected by the pathology. Roughly 25 cm of ileum and 80 cm of jejunum were resected. Bowel continuity was restored with an ileo-ileal and a jejuno-jejunal anastomosis. Almost 700 cl of pus were drained and the peritoneal cavity was washed with 10 L of saline solution. The postoperative recovery was uneventful and the patient was discharged 8 days later. There were no signs of malignancy in the resected intestine. |
A 55-year-old incarcerated male presented to the emergency room with a two-week history of left-sided scrotal pain and swelling.\nThis patient had a history of prostate cancer and high-grade urothelial bladder cancer. His prostate cancer was diagnosed 15 years prior to the current presentation, managed with radiation, and had since remained stable. The patient's high-grade, high-risk bladder cancer was diagnosed two years prior to current presentation. At the time, the patient initially presented with hematuria. A CT urogram performed at the time revealed a nonspecific bladder mass. Biopsy of the mass confirmed urothelial carcinoma. The patient's therapy course included a transurethral resection of the bladder tumor (TURBT), intravesical mitomycin, interferon alfa-2b, and intravesical BCG therapy. He received a total of seven intravesical injections of 50 mg live BCG per injection (350 mg cumulative dose) over the course of 18 months.\nThe patient's other significant medical history included rheumatoid arthritis, for which the patient was on weekly methotrexate and daily tofacitinib treatment. Both medications were discontinued on admission.\nThe patient developed scrotal pain two weeks prior to admission. He initially presented to the emergency department with this pain. At that time, he was diagnosed with acute bacterial epididymitis and was prescribed a course of ciprofloxacin. He had no improvement in his symptoms despite this treatment. Over the subsequent days, he reported experiencing intermittent chills and night sweats. He denied any penile discharge or any history of a sexually transmitted disease.\nEvaluation of the testicle with ultrasound revealed multiple diffuse nodular areas of hypoechogenicity (Figure , ), as well as marked hypervascularity involving all of the left-sided structures (Figure , ). Routine blood and urine cultures were negative, and mycobacterial blood and urine cultures, as well as bladder biopsy, were pending as of this writing.\nAntituberculous therapy was started based on the following considerations regarding the patient's presentation. The lack of observed response to ciprofloxacin therapy decreased the likelihood of uncomplicated bacterial orchitis. We considered this patient's history of bladder cancer involving multiple BCG treatments and the increased likelihood of BCG orchitis. Possibly, his immunocompromised state secondary to the malignancy, in combination with mitomycin chemotherapy and immunomodulatory rheumatoid arthritis treatment, could have increased his susceptibility to a mycobacterial infection. Finally, as ultrasound findings were characteristic of BCG orchitis, the patient was promptly initiated on a combination therapy with levofloxacin, rifampin, isoniazid, and ethambutol. The patient showed marked improvement in pain control and testicular swelling over the following weeks. |
A 26-year-old G2 P2 L1 pregnant full-term female was referred to our hospital for delivery. She gave a history of rapid weight gain, hyperpigmentation, fatigue and weakness, with all complaints beginning in the third trimester. Her first child was born out of full-term cesarean section under spinal anesthesia, the indication being cephalopelvic disproportion and recovery was uneventful. She weighed 60 kg before pregnancy and had gained 30 kg in this pregnancy, the majority of which was gained in the last trimester. She also complained of hyperpigmentation over the arms and the back. No other positive medical or surgery history was elicited.\nOn examination, she weighed 90kg and had abdominal striae but the colour could not be discriminated because of her dark complexion. She had thin skin, moon facies and buffalo hump too. Her blood pressure was 130/80 mmHg, and her pulse rate was 106/min with a normal systemic examination. Her complaints and cushingoid features demanded serum cortisol levels. We also sent her blood for thyroid function tests and sugars apart from other routine examination.\nThyroid function tests, blood sugars and rest of the hematology were normal. However, serum cortisol was raised which was upto 26.33 μg/dl. Given her clinical features and high serum cortisol, a presumptive diagnosis of Cushing's syndrome was made. But, the case, because of its rarity demanded further investigations to discern the etiology. The most common cause of Cushing's syndrome in pregnancy lies in the adrenal glands; either an adenoma or adrenocarcinoma. An abdominal ultrasonography was performed to rule out adrenal pathology, which was negative. We then sent her urine for urinary free cortisol (UFC) and serum for ACTH levels.\nHer values were:\nThese levels were very high for normal pregnancy and her raised ACTH levels pointed toward a pituitary cause.[] But, as the patient was brought in full term, we had to accept the patient without further investigations for cesarean section.\nElective cesarean section was planned for our full-term patient in view of previous cesarean and cephalopelvic disproportion. Challenges for anesthesiologists were her weight, with central obesity and fat deposition over the back, buffalo hump and heavy jaw, making regional as well as general anesthesia difficult. Anesthesia plan of action was spinal anesthesia with 1.5 ml 0.5% hyperbaric bupivacaine. Difficult mask ventilation and intubation were anticipated and preparations were made accordingly. The patient received antacid prophylaxis preoperatively.\nSpinal anesthesia was given with a 26G Quincke's spinal needle in the L3-L4 interspace at the first attempt but at a greater depth than always. Obstetricians faced difficulty because of excessive fat deposition and adhesions from previous cesarean. A baby weighing 2.5 kg was delivered with an APGAR score of 9. The patient's electrocardiogram, blood pressure and saturation were monitored through out the procedure and were normal except for sinus tachycardia. Post-operative analgesia was given in the form of diclofenac suppository 100 mg per rectal. The rest of the intra- and post-operative period was uneventful.\nHer placenta was sent to histopathology for examination of any hyperplasia of cells. Two days post operatively the patient was scheduled for magnetic resonance imaging (MRI) of the brain to visualize sella tursica. Her placenta showed a normal picture; however, her MRI brain revealed a 2.7mm nonenhancing foci in the anterior pituitary, which was suggestive of pituitary microadenoma (accompanying MRI picture).\nIn concise, our patient had cushingoid features associated with hypercortisolism, increased ACTH levels and the MRI brain showed pituitary microadenoma. Thus, in fact, she was a case of Cushing's syndrome, the cause of which was Cushing's disease. |
A 52-year-old man presented with left facial dysfunction and went to a local hospital in 2016. Brain magnetic resonance imaging (MRI) at the local hospital revealed a mass in the saddle area. The tumor was 6.3 cm × 5.8 cm × 4.5 cm. The patient was referred to a tertiary hospital and was diagnosed with a hypophysoma. He received pharmacotherapy and his facial numbness went into mild remission. In 2017, his MRI showed that the tumor grew from 6.3 cm to 7 cm. He continued treatment with medicine. The patient felt that his eyesight diminished further, and in April of 2018, he was admitted to hospital. The patient had no history of trauma, no family history of any hereditary illness, and his neurological examinations were normal.\nPreoperative computer tomography (CT) and MRI showed a huge tumor in the sellar area. A CT scan revealed a hyperintense mass with an indistinct boundary located in the saddle area, sphenoid sinus area, and left temporal lobe; the tumor demonstrated expansive growth ( and ). MRI showed a 7 cm × 6 cm × 5 cm giant irregular mass in the left side saddle, sphenoid sinus, and medial temporal lobe. The tumor presented as non-uniform hyperintense signal on T1-weighted images and uniform hyperdense signal on T2-weighted images ( and ). Based on preoperative imaging results, the provisional diagnosis was a malignant tumor of the sellar region. A neurosurgeon removed the tumor by transcranial endoscopy. During tumor removal, the surgeon found that the tumor tissue was soft and had a fish-like appearance, with a rich blood supply. The tumor was completely removed piece-by-piece under an endoscope. Postoperative CT imaging on the first day after surgery showed that the tumor was resected (–). The patient accepted radiation therapy at the local hospital 1 month later (total dose, 50 Gy; 2 Gy once a day for 4 weeks). At a follow-up investigation 1 year after tumor resection, the patient’s condition was generally good. |
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